JUNE 4-7th 2008
1. Winner of the Young Investigator’s Award
Atrial Fibrillation Doubles Mortality in Adults with Congenital Heart Disease: A Population Based Study from 1983 to 2005.
Bouchardy J, Therrien J, Pilote L, Ionescu-Ittu R, Martucci G, Marelli AJ- MAUDE Unit, MUHC, Montreal, QC, Canada
Background: Although ventricular arrhythmias have been extensively studied in adults with congenital heart disease (ACHD), data
examining the impact of atrial arrhythmias (AA) on outcomes of ACHD patients are lacking. Our objectives were to determine
prevalence of AA in an ACHD population and to compare mortality and hospitalization rates in those with and without AA.
Methods: The study population consisted of all patients > 18 years in year 2005. A congenital heart disease diagnosis was assigned to
each patient using ICD-9 diagnostic codes in administrative databases of Quebec from 1983 to 2005. Patients with AA are those
diagnosed with an ICD-9 code for atrial fibrillation or intra-atrial-reentry-tachycardia over the 18 years study period. Prevalence of
AA was measured in year 2005 as AA cases per 1,000 ACHD patients as well as in lesion and age-specific groups. We report the
crude rates for mortality (deaths/1,000 population) and hospitalization (hospitalization days / 1000 person-days) in year 2005 in those
with and without AA and the corresponding rate ratios (RR) after direct standardization for age group and CHD severity.
Results: In a population of 38,428 ACHD patients, 5,812 (150/1,000 ACHD population) had AA. Prevalence of AA varied from
49/1,000 in patients with ventricular septal defect to 274/1,000 in those with transposition of the great arteries, and from 28/1,000 in
age group 18-35 years to 394/1,000 in those aged > 65 years. Crude mortality rate was 55 /1,000 in the AA group versus 9/1,000 in
the non-AA, corresponding to a standardized RR of 2.14 (95% confidence interval 1.81-2.52). Crude hospitalization rate was 5.06
days /1,000 person-days in the AA group versus 0.81 /1,000 in the non-AA population, corresponding to a standardized RR of 3.16
(95% confidence interval 3.06-3.26).
Conclusion: In this large ACHD population study, 15% of patients had AA. The presence of atrial arrhythmias in ACHD patients
triples hospitalization rates and doubles mortality.
2. Potential Therapeutic Role of ACE Inhibitors in Repaired Tetralogy Of Fallot and Pulmonary Regurgitation - The
Appropriate Study (ACE Inhibitors for Potential Prevention of the Deleterious Effects of Pulmonary Regurgitation In Adults
with Repaired Tetralogy of Fallot).
Babu-Narayan SV, Uebing A, Davlouros PA, Path MK, Davidson S, Bayne S, Dimopoulos K, Pennell DJ, Gibson DG, Kilner PJ, Li
W, Gatzoulis MA- Royal Brompton Hospital, London, United Kingdom
Background: We aimed to examine whether Angiotensin-Converting-Enzyme (ACE) inhibition could improve right ventricular
(RV) function in patients with pulmonary regurgitation (PR), a cause of late morbidity and mortality in repaired tetralogy of Fallot
Methods: Seventy-two patients were recruited, of whom 64, contributed to the final analysis. These stable patients with rTOF and
moderate or severe PR were randomised to 6 months of ramipril or placebo, uptitrated to 10mg once daily, in a double-blinded,
placebo-controlled study. All patients underwent cardiovascular magnetic resonance, echocardiography, neurohormonal analysis, and
cardiopulmonary exercise at baseline and at six months follow up.
Results: Comparing changes from baseline to 6 months between ramipril and placebo, there were no significant differences in right
ventricular (RV) or left ventricular (LV) ejection fraction (EF), but there was significant improvement in both RV and LV long-axis
shortening (RV; 2.3±3.8 vs. 0.02±2.7mm;P=0.017, LV; 1.9±4.5 vs. -0.2±3.7mm;P=0.030). There was no significant change in PR
fraction, biventricular volumes, neurohormones or objective cardiopulmonary exercise.In the subgroup of patients with RV restrictive
physiology (n=27) there was significant improvement in LV end-systolic volume index and LVEF (-2.4±5.0 vs 2.7±3.6 ml/m2;
P=0.005, 2.5±5.0 vs. -1.3±3.5%; P=0.03).Ramipril was well tolerated and there were no deaths or major adverse events.
Conclusion: Ramipril may have a role for the management of PR after rTOF with trends to improvement in RV and LV function and
treatment was well tolerated. Ramipril should be considered for patients with a restrictive RV, in whom LV function improved.
Our findings should form the basis for larger, systematic studies of vasodilator therapy in this at risk patient group. ISRCTN:
3. Beyond Late Gadolinium: Quantifying diffuse myocardial fibrosis and its role in ventricular dysfunction in adult congenital
Broberg C, Chugh S, Sahn D, Jerosch-Herold M - Oregon Health and Science University, Portland, OR, United States
Background: The etiology of ventricular dysfunction in adult congenital heart disease (ACHD) is not well understood. Background
studies suggest that diffuse myocardial fibrosis exists and is a potential therapeutic target. A reliable method for detection and
quantification of diffuse fibrosis would facilitate study of the problem. We have perfected an MRI-based “fibrosis coefficient” to
quantify diffuse fibrosis, namely the amount of extracellular space within the myocardium. We sought to quantify diffuse fibrosis in
Methods: Consecutive patients were prospectively studied with cardiac MRI (Philips Achieva) to quantify ventricular volume and
function. The fibrosis coefficient was quantified by comparing signal intensity curves for the myocardium and blood pool before and
after administration of gadolinium (0.15 mmol/kg). Results were compared to normal controls (N=10) and patients with acquired
heart failure (N=4).
Results: Twenty-five ACHD patients (age 37±12 years) were studied (8 with tetralogy of Fallot, 6 with Eisenmenger syndrome, 5
with transposition of the great arteries, and 6 miscellaneous). The fibrosis coefficient was significantly elevated in ACHD patients
compared to controls (31±6% vs. 26±2%, p=0.003) and was comparable to patients with acquired heart disease (31±6%). Values were
highest in Eisenmengers (33±6%, p=0.002) and transposition patients (34±6%, p<0.001). The fibrosis coefficient correlated with end-
diastolic volume index (R=0.52, p=0.03). There was also a strong correlation with systemic ventricular ejection fraction in the
Eisenmenger patients (R=-0.79, p<0.01). Similar trends were observed for the transposition patients. The tetralogy of Fallot patients
had lower values of the fibrosis coefficient. No large areas of late gadolinium enhancement were present in any patient.
Conclusion: ACHD patients have evidence of diffuse, microscopic myocardial fibrosis similar to patients with acquired heart failure.
This supports the theory of ACHD in general as a form of heart failure. The fibrosis coefficient may facilitate much needed studies
on the mechanism and treatment of myocardial fibrosis in these patients.
1. Restrictive Lung Disease is Common in Adults Following Repair of Congenital Heart Disease.
Earing M, Bartz P, Danduran M, Deatsman S, Gorentz J, Morrissy M, Koppein D, Romfh A, Sowinski J, Biller J - Medical College of
Wisconsin, Milwaukee, WI, United States
Background: Following repair of congenital heart disease (CHD), adult patients continue to be at risk for the development of late
complications, both cardiac and non-cardiac. These late complications include reduced aerobic capacity and restrictive lung disease.
However, the true incidence of restrictive lung disease in adults following repair of CHD remains unknown. We sought to determine
the incidence of restrictive lung disease (RLD) in adults following repair of CHD.
Methods: Records were reviewed of 100 consecutive patients (45 male; 55 females) evaluated in the Adult Congenital Heart Disease
program with CHD who underwent treadmill exercise stress testing with spirometry (mean age 33 + 12 yr). Connective tissue
disorder and muscular dystrophy patients were excluded. Clinical data was obtained by retrospective chart review. Results of
spirometry and flow/volume loops were reviewed and categorized by a single blinded observer.
Results: The most common diagnoses for the cohort was coarctation of the aorta (n=19). Additional defects included conotruncal
abnormalities (n=17), single ventricle (n=9), left heart lesions (n=8), atrial or ventricular septal defect (n=8), D-TGA (n=8), Ebstein’s
anomaly (n=7), L-TGA (n=6), and other (n=18). For the cohort, 21 patients had had no previous surgical repair. This group was
comprised of L-TGA, bicuspid aortic valves, Ebstein’s anomaly, and mild coarctation of the aorta. Of the 79 patients who underwent
previous cardiac surgery, 34 patients had 1 surgical procedure, 23 patients had 2 surgeries, 15 patients had 3 surgeries, and 7 patients
had undergone 4 surgeries. Overall, 45% had abnormal exercise capacity defined by exercise duration being less than 80% predicted.
Predictors of abnormal exercise capacity included history of single ventricle (p<0.001), heart rate reserve (p<0.001), number of
previous surgeries (p <0.002), length of follow-up (p<0.05), and spirometry suggestive of RLD (p <0.03). Based on spirometry and
flow/volume loops, 50 patients were classified as normal, 44 patients had patterns suggestive of RLD, 4 suggestive of mixed
(obstructive and restrictive), and 2 indeterminate. Patients with single ventricles were most likely to have spirometry suggestive of
RLD (n=8, 89%), followed by patients with conotruncal abnormalities (n=12, 71%). The most significant predictors of RLD by
spirometry included history of previous repair (p< 0.001) and a history of amiodarone therapy (p< 0.008). Age at initial surgery,
length of follow-up, tobacco use, previous thoracotomy, and history of systemic ventricular dysfunction were not significant. Overall,
61% (n=48) of patients who had undergone previous repair had spirometry suggestive of RLD compared to 14% (n=3) of the patients
who had unrepaired CHD (p<0.001). Of the 3 unrepaired patients with spirometry suggestive of RLD, all 3 had previously been
treated with amiodarone. Overall, compared to patients with normal lung function, patients with spirometry suggestive of RLD had
lower VO2 (p<0.001), VO2 index (p<0.002), and exercise duration (p<0.004).
Conclusion: Adult patients who have undergone previous repair of CHD often have decreased exercise capacity and RLD suggested
by spirometry. RLD likely contributes to exercise intolerance following repair in adults with CHD. Further studies are needed to
evaluate the relationship between exercise intolerance and the degree of RLD.
2. A Contemporary Prospective on Eisenmenger Syndrome in Adults: A Multicentre Study.
Silversides CK, Connelly M, Mercier LA, Therrien J, Vonder Muhll I, Beauchesne LM, Burggraf G, Dias B, Gordon E, Kiess M,
Rodes J, Tam J, Alharbi M, Oechslin EN - Canadian Adult Congenital Heart Network, Canada
Background: Eisenmenger syndrome (ES) is a systemic disease with both cardiac and non-cardiac complications. The frequency and
time at which these adverse events occur have not been well described. The objective of this study is to systematically examine late
complications in a contemporary adult cohort of patients with ES.
Methods: Thirteen primary Canadian adult congenital heart disease referral centers participated in the study. Adults (≥ 18 yrs)
with ES actively followed in the clinics were included. A retrospective chart review was performed at each site. Adverse events were
recorded and included: 1) bleeding complications primarily necessitating medical consult or intervention, 2) thromboembolic
complications (cerebral vascular events or pulmonary thromboembolism), 3) heart failure requiring therapy, 4) arrhythmias requiring
treatment and 5) infections including pneumonias and cerebral abscesses. Medical therapy was recorded. Medications specific for
pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, phosphodiesterase antagonsits and prostacycline
Results: In total, 214 adults with ES (69% female, mean age 42 ± 12 yrs) were included. The most common cardiac diagnoses were
ventricular septal defects (n=89), atrioventricular septal defects (n=54) and atrial septal defects (n=31). Eleven percent of patients
were taking oral anticoagulants, 15% were taking acetylsalicylic acid and 28% were on medical treatment specific for PAH. Fifty
percent of patients had significant functional limitations (NYHA functional class III or IV) and of these patients, 36% (39/107) were
on medical therapy for PAH. The most frequent adverse events were related to bleeding complications, most commonly hemoptysis
(31%, mean age at presentation 33 ± 12 yrs). Thromboembolic events were less common (15%, mean age at presentation 31 ± 10
yrs). Heart failure developed in 20% (mean age at presentation 32 ±18 yrs) of this cohort and arrhythmias (SVT n=15 and VT n=9,
not mutually exclusive) in 10% (mean age at presentation 38 ±14 yrs). Pneumonia was documented in 51 patients (mean age 27 ± 17
yrs) and cerebral abscess in 4 patients.
Conclusion: Complications occur frequently in adult patients with ES during their 3rd decade of life. Bleeding, infections and heart
failure are the most common adverse events. The use of medical therapy for patients in functional class III/IV is likely underutilized.
This study highlights the need for continued surveillance and timely consideration of medical therapy specific for PAH in select
3. Identifying New Genetic Forms of Tetralogy of Fallot.
Costain N, Marshall S, Christian S, Oechslin EN, Kovacs A, Greenwood C, Chow E, Silversides CS, Bassett A- Centre for Addiction
and Mental Health and Adult Congenital Heart Disease Program, Peter Munk Cardiac Centre, Toronto General Hospital, University
of Toronto, Toronto, ON, Canada
Background: Among patients with tetralogy of Fallot (TOF), there is clinical heterogeneity and likely considerable underlying
genetic heterogeneity. Approximately 8-15% of cases have 22q11.2 Deletion Syndrome (22qDS) associated with 1.5 - 3 megabase
(Mb) deletions (copy number changes) on chromosome 22q11.2. In most other patients, no genetic basis for TOF has been identified.
Methods: We have recruited patients with TOF at our clinic (TCCCA) for genetic studies. We have identified a "Syndromal"
subgroup (15.5%) of patients, not previously described, who have extracardiac features suggesting a possible genetic syndrome but
who do not have 22qDS or karyotypic abnormalities. In a pilot study, we used an Affymetrix 250K NspI SNP array to investigate
copy number variants (CNV) in 29 unrelated adults with TOF, 14 who met our clinical Syndromal criteria, but who did not have
22qDS or karyotypically detectable anomalies, and 15 Non-syndromal patients. We compared results to those for 1190 population
controls and confirmed positive results with quantitative PCR (qPCR).
Results: We found 3 of 14 (21.4%) Syndromal and no Non-syndromal subjects had large scale CNV (>1Mb) that were not present in
1190 controls. These were: a 4.3Mb 22q11.2 duplication likely similar to those in recently reported cases, and two novel CNV: a 1.4
Mb 1q41 deletion and a 4.7Mb 13q13 deletion.
With respect to novel CNV of a smaller size (10kb - 1Mb), there were 11 in the Syndromal group and 12 in the Non-syndromal
group. One of these was a "recurrent" CNV present in each group, a gain (duplication) involving an overlapping 51kb (in the
Non-syndromal subject) and 75kb (in the Syndromal subject) region on 5q22 containing an interesting gene with cardiac expression.
Only 4 of 1190 (0.4%) control subjects had an overlapping CNV. qPCR validated the CNV in all 5 of these cases.
Conclusion: These pilot results support the likelihood that careful clinical assessments and advanced molecular methods will identify
novel genetic forms of TOF and thereby genes that are important to cardiac development.
4. Risk factors for mortality and functional deterioration in adults with single ventricle: a five year follow-up study.
Larsson D, Thilen U, Holm J, Meurling C- Department of Cardiology, Lund University, Sweden
Background: Adults with single ventricle malformations suffer from significant morbidity and mortality, and require frequent
clinical evaluation in order to optimize treatment. However, it is yet unclear what factors are most important for the long-term
prognosis. We conducted a subgroup analysis of the patients with single ventricle included in the Euro Heart Survey on adult
congenital heart disease in Europe, aiming to assess the prognostic powers of clinical parameters and symptoms.
Methods: 198 patients with single ventricle (median age 23 years), who visited the outpatient clinics of one of the participating
centers in 1998, were included retrospectively and followed for a median period of 5 years. Underlying diagnoses were Tricuspid
atresia (n = 107), other systemic left ventricle (n = 44), systemic right ventricle (n = 14) and indeterminate morphology (n = 33).
Clinical variables were recorded at baseline and during follow-up.
Results: At baseline, 40% of patients were in New York Heart Association (NYHA) functional class I, and 70% had normal systemic
systolic ventricular function (ejection fraction). Supraventricular arrhythmias and systemic atrioventricular valvular regurgitation
were present in 46% and 56%, respectively. Supraventricular arrhythmias were significantly less common in those with a total
cavopulmonary connection (24% vs. 52%; p < 0.005).16 (8%) of patients died during follow-up. No particular anatomy or palliation
was overrepresented in this group, and neither systolic ventricular function nor NYHA class could predict those at risk. However,
systemic atrioventricular valvular regurgitation significantly increased the risk of death (p < 0.01), and there was a trend towards
higher prevalence’s of supraventricular arrhythmias (68%; p = 0.07) among the deceased. 13 (81%) were in NYHA class > I.
20 patients deteriorated in terms of NYHA class during follow-up. No relation was found to baseline ventricular function, arrhythmias
or atrioventricular valvular regurgitation.
Conclusion: The poor prognostic power of systolic ventricular function demonstrated in the study suggests that systolic heart failure
in adults with single ventricle malformations is less important a marker of cardiac function than previously thought. Arrhythmias and
cardiac problems such as atrioventricular regurgitation may better identify those at risk of deterioration.
5. Improvement in Left Ventricular Function after Pulmonary Valve Replacement in Patients with Tetralogy of Fallot.
Tanous D, Subira LD, Caldarone C, Oechslin EN, Silversides CS- Adult Congenital Heart Disease Program, Peter Munk Cardiac
Centre, Toronto General Hospital, University of Toronto, Toronto, ON, Canada
Background: Left ventricular (LV) dysfunction can develop in patients with repaired tetralogy of Fallot (rTOF). The LV dysfunction
is felt to be due to, at least in part, adverse ventricular-ventricular interactions secondary to the abnormal dilated right ventricle (RV).
The purpose of this study is to determine whether pulmonary valve implantation (PVR) and subsequent RV remodeling, is associated
with improvements in the function of the LV.
Methods: Pre and post-operative echocardiograms of consecutive patients with rTOF undergoing PVR at a single centre between
August 2003 and October 2006 were reviewed. Patients were excluded if: a) they were not in sinus rhythm at the time of either
examination, b) they had inadequate echocardiographic images or c) they were undergoing concomitant tricuspid, aortic or mitral
valve surgery. RV and LV function were measured echocardiographically using the myocardial performance index (MPI). The MPI,
a parameter for global ventricular function, measures both systolic and diastolic function of the ventricles. LV ejection fraction was
calculated using Quinones method. RV end-diastolic diameter was measured on the apical 4-chamber view at 1/3 of the distance from
the annulus to the apex. Comparisons between pre- and post-operative data were made using Wilcoxon signed-rank test.
Results: Twenty patients (55 % males, mean age 32 ± 10 years) with rTOF undergoing PVR were included. The mean age at initial
intra-cardiac repair was 6 ± 3 years and in 15 patients the repair included a transannular patch. The primary indication for surgery was
significant pulmonary regurgitation with associated RV dilation, with symptoms present in 11 of the 20 patients (8 were in NYHA
class II and 3 were in NYHA class III). Pre-operative echocardiograms were performed 295 ± 154 days (range 3-626 days) prior to
surgery and post-operative echocardiograms were performed 493 ± 277 days (range 83-1077 days) following surgery. The pre-
operative RV MPI was 0.30 ± 0.11 and the LV MPI was 0.53 ± 0.14, indicating baseline biventricular dysfunction. The RV end-
diastolic diameter decreased from 52 ± 7 mm pre-operatively to 43 ± 10 mm post-operatively (p < 0.001). Improvements were evident
in both the RV MPI (0.30 ± 0.11 vs. 0.23 ± 0.13, p = 0.04) and the LV MPI (0.53 ± 0.14 vs. 0.45 ± 0.14, p = 0.02) after surgery.
There was no significant change in LVEF (0.54 ± 0.10 vs. 0.56 ± 0.07, p = 0.32).
Conclusion: In patients with rTOF, the global function of the left ventricle shows improvement after pulmonary valve implant.
Reductions in RV size and subsequent improvements in ventricular-ventricular interactions are one possible explanation for this
6. Sexual Dysfunction in Men with Congenital Heart Disease: Under-Reported and Rarely Discussed.
Cook S, Arnott L, Nicholson L, Cook L, Hickey J, Crumb S, Daniels C- Nationwide Children's Hospital, Columbus, OH, United
Background: There has been a tremendous focus on gynecologic health with the increasing number of women with congenital heart
disease (CHD) now reaching childbearing age. Conversely, experience in providing similar sexual health screening to men with
CHD is lacking. The purpose of this study was to identify sexual health issues in men with CHD.
Methods: An anonymous survey was distributed to 86 men in the Adult CHD clinic. Age, type of CHD, level of education,
medication history, detailed sexual history including cardiovascular symptoms with sex, impotence and/or erectile dysfunction (ED)
and willingness to discuss ED with a physician was obtained. A logistic regression analysis was performed to analyze the
relationship between medications and ED.
Results: Eighty-six men completed the survey. The mean age was 33 ± 10 years. There were 19 men with simple CHD, 34 with
CHD of moderate severity, 18 with complex CHD and 15 who did not know their CHD. Most men graduated from high school
(51%) and one-third had a college degree (34%). The prevalence of ED was 33%. As age increased, the odds of reporting ED
increased (p=0.02). Fifty-three men were treated with cardiac medications including beta-blockers (BB, n=35) and angiotensin-
converting enzyme inhibitors (ACEI, n=21). Among all men, those on BB were 3.8 times more likely to report cardiovascular
symptoms (p=0.038), compared to those on no medications and 4.3 times more likely to report symptoms than those on BB and
ACEI (p=0.035). Among all men, those on BB were 80% more likely to report symptoms of ED compared to those on no
medications (p=0.011). Twenty men (23%) attributed these symptoms to their underlying CHD. Most men (84%) reported they
would discuss issues of ED with their cardiologist.
Conclusion: Sexual health issues, notably ED, are prevalent in young men who have CHD. Although an emotional component may
contribute to these symptoms, certain medications may exacerbate symptoms (BB) while others may have a protective effect
(ACEI). However, questions and fears regarding sexual health may not be expressed voluntarily. Therefore, discussion of this
sensitive but important topic should be initiated with the physicians and nurses who care for this unique population of patients.
7. Prevalence and consequences of arrhythmias in adults with single ventricle: a five year follow-up study.
Larsson D, Thilen U, Holm J, Meurling C- Dept of Cardiology, Lund University, Sweden
Background: Arrhythmias are common in patients with single ventricle malformations and lead to significant morbidity and
mortality. Whereas much is known about arrhythmias in children with single ventricle, their importance late after Fontan operation
is less well studied. We conducted a subgroup analysis of the patients with single ventricle included in the Euro Heart Survey on
adult congenital heart disease in Europe, aiming to assess the relationships between arrhythmias, symptoms and prognosis.
Methods: 198 patients with single ventricle (median age 23 years), who visited the outpatient clinics of one of the participating
centres in 1998, were included retrospectively and followed for a median period of 5 years. Underlying diagnoses were tricuspid
atresia (n = 107), other systemic left ventricle (n = 44), systemic right ventricle (n = 14) and indeterminate morphology (n = 33).
Clinical variables were recorded at baseline and during follow-up.
Results: At baseline, 96 (49%) of patients had at least one arrhythmia. 91 (46%), 14 (7.1%), 3 (1.5%) and 1 (0.5%) patients had
supraventricular arrhytmias (SVA), atrioventricular block, sinus node dysfunction and ventricular arrhythmias, respectively. Patients
with SVA were significantly older (median 25 vs. 21 years; p < 0.001). SVA were significantly less common in those with a total
cavopulmonary connection compared to those with other types of palliations (24% vs. 52%; p < 0.005). 26 patients that did not have
SVA at inclusion developed SVA during the follow-up period. 16 (8%) of patients died during follow-up. Of these, 13 either had
SVA at baseline or developed SVA during follow-up. Though not statistically significant, there was a trend towards higher
prevalence’s of supraventricular arrhythmias among the deceased (p = 0.07). 20 patients deteriorated in terms of New York Heart
Association (NYHA) class during follow-up. Subjects that had SVA at inclusion or developed SVA during follow-up were over-
represented in this group (n = 17; p < 0.05).
Conclusion: The present study shows that arrhythmias are very common in adults with single ventricle malformations. The vast
majority of these are of supraventricular origin. Furthermore, many patients develop new supraventricular arrhythmias late after the
Fontan operation. These arrhythmias are associated with risk of functional deterioration and also seem to increase mortality. Finally,
the study confirms the superiority of the total cavopulmonary connection as far as arrhythmias are concerned.
8. Catheter Ablation of Ventricular Tachycardia in Corrected Tetralogy of Fallot.
James S, Bourke JP, O'Sullivan JJ - Freeman Hospital, Newcastle Upon Tyne, United Kingdom
Background: Ventricular tachycardias (VT) and cardiac sudden death are recognized late complications following correction of
tetralogy of Fallot (ToF). VT can occur in the setting of severe pulmonary regurgitation (PR) and failing right ventricle (RV), or as a
consequence of scarring in a hemodynamically stable state. ICD implantation is the preferred treatment in most scar-related VT
types. The young patient age and RV origin of VT make it desirable to abolish VT instead of implanting ICD if it is safe/ feasible.
We describe the clinical outcome for ToF patients with recurrent VT referred to our centre for catheter ablation since 1997.
Methods: Patients initially underwent hemodynamic assessment by combination of echo, MRI and/or cardiac catheterization. Those
felt to be appropriate went on to have catheter ablation performed, using a combination of substrate/scar mapping in sinus rhythm,
timing map during VT and pace-mapping techniques.
Results: 17 patients were assessed, 4 patients had significant PR and underwent pulmonary valve replacement with interoperative
cryoablation alone, 3 patients had syncopal VT or VF as their index arrhythmia and underwent ICD implantation, 10 patients
underwent radiofrequency ablation, 1 was performed following pulmonary valve replacement and cryotherapy, 2 required repeat
procedures during the same admission, 9 patients were had successful short outcomes and have been free of VT since (follow up
duration range 0.5 to 10 years, mean 3.4 yrs), 1 patient was not possible to suppress VT despite repeat procedures and subsequently
had ICD implant (this patient was noted to have severe hemodynamic impairment prior to ablation).
Conclusion: While ICD implantation is needed for syncopal ventricular arrhythmias, it can be avoided in majority of ToF patients
with VT. Providing there is reasonable hemodynamic function catheter ablation should be considered standard treatment for
recurrent VT in ToF as this offers excellent short and long-term outcome.
9. The Assessment of Regional Right Ventricular Function in Patients After Repaired Tetralogy of Fallot: Myocardial Strain
Measurement by Speckle Tracking Echocardiography in Comparison with Tagging by Cardiac Magnetic Resonance
Lyseggen E, Wald R, Redington AN, Silversides CK, Siu SC, Provost Y, Oechslin EN- Adult Congenital Heart Disease Program,
Peter Munk Cardiac Centre, Toronto General Hospital, University of Toronto, ON, Canada, and Department of Cardiology,
University of Oslo, Norway
Background: Tissue Doppler echocardiography (TDE) provides non-invasive assessment of right ventricular (RV) regional
myocardial strain (deformation), but is limited by angle dependency. Speckle tracking echocardiography (STE) is a new non-
invasive method for angle independent assessment of regional myocardial function as it is not necessary to align the ultrasonic beam
and the direction of myocardial motion. We speculated that this method may prove superior to TDE particularly in patients with
congenital heart disease who commonly have variations in cardiac anatomy and/or position. In this study, we introduce STE as a
novel method for assessment of RV free wall strain in repaired TOF and we validate these results against RV strain as measured by
cardiac magnetic resonance imaging (CMR) tagging.
Methods: We prospectively enrolled 21 consecutive patients (mean age 33±11 years) with repaired TOF (age at repair 8±7 years).
Echocardiography with STE and CMR with tagging were performed on the same day. RV long-axis recordings (4-chamber view)
were obtained by conventional 2D grayscale echocardiography. The following parameters were adjusted for optimal speckle quality
of the recordings: sampling rates were 81±31 frames per second, sector width was kept as narrow as possible and depth was adjusted
to be as shallow as possible. One patient was excluded due to poor RV image quality. Complete RV free wall STE datasets at base,
mid and apical segments were obtained from 20 patients and analyzed offline with EchoPac v6.0, GE Vingmed. No segments were
excluded from analysis. CMR grid tagging images in the 4-chamber view were obtained using a commercially available scanner and
data were analyzed with Harmonic Phase Imaging (HARP v 4.0, Diagnosoft Inc.).
Results: There was a good correlation (r=0.75, y=0.98x-1.4, P=0.0001) and good agreement (mean difference -0.89±2.5%) for
average RV free wall strain analysis by CMR tagging and STE. Average RV free wall strain by CMR tagging was -20.7±2.9% and -
21.23±4.0% for STE. Mean strain values by segment obtained by CMR tagging and STE respectively were: (1) base -19.6±4.6%
and -20.9±6.3%, (2) mid -21.4±4.3% and -22.2±5.2% and (3) apical -21.0±4.8% and -20.9±5.5%. At segmental levels, strain by
STE was correlated with CMR tagging values (r=0.50, P<0.0001).
Conclusion: The present study demonstrates that regional RV free wall strain can be measured accurately by STE and is feasible in
a bed-side clinical setting, suggesting a new echocardiographic approach for quantification of regional RV systolic function in
patients with repaired tetralogy of Fallot. Average RV free wall strain correlates better than strain at the segmental level, when
assessed by STE and CMR tagging.
10. Cardiovascular events in Marfan syndrome, Follow up and treatment of 152 Swedish Marfan patients.
Moberg K, Thilén U, Holm J- Kardiologiska Kliniken, UMAS Malmö, Sweden
Background: Marfan syndrome (MFS) can lead to life threatening cardiovascular complications such as dilatation and dissection of
the aorta by medial aortopathy. The Swedish GUCH register (Grown up Congenital Heart Diseases) is a national register for patients
from specialized GUCH units. The aim was to analyze data from GUCH registered MFS patients to evaluate age at diagnosis,
treatment with beta-blocker, clinical follow-up (F/U), surgical treatment and quality of life (QoL).
Methods: Unidentified data of all 152 patients (69 women and 83 men, mean age 37 years, range 18-73 years), diagnosed with MFS
in the GUCH register was retrieved in June 2007. Data included gender, age at diagnosis and additional diagnoses. 14 patients with
insufficient data were excluded except for additional diagnoses and gender.
Clinical variables were blood pressure, current medication, surgery, and QoL. Medical F/U was evaluated from dates and number of
visits to a cardiologist and performed examinations with EKG, TTE, TEE, CT-scan or MRI. Pregnancy data and maximal aortic
diameter was also retrieved. F/U interval was 2002 to 2007.
Results: Women with MFS were older, mean age 40 vs. 34 years, (p = 0,002) and diagnosed at an older age. At time of diagnoses
mean age was 22 years (range 0-71) with an older age among women: 28 vs.19 years (p=0.005). Five patients died in these five
years. Mean F/U time in the GUCH register was 3,2 years. Additional diagnoses were dilatation of the ascending aorta (n = 25);
aortic dissection (n = 14); aortic valve insufficiency (n =10) and mitral valve insufficiency (n =9). Average blood pressure was
123/73 mmHg. 50 patients were treated with beta-blockers and 27 with warfarin. Average number of visits to a cardiologist was 2.4
and average time between visits was 2.3 years. Between 2004 and 2007, 90 patients had a TTE, 33 patients had a CT-scan or MRI
and 14 patients had both. 50 patients had neither TEE/TTE nor CT-scan or MRI. Mean aortic diameter was 33 mm (range 19-60mm)
and mean diameter at time of surgery was 48mm (range 43-56mm). Valve or aortic graft surgery was performed in 22 patients. Of
these, 13 had aortic graft surgery and 14 had valve surgery, 5 patients underwent both. Surgery was more common among men (22
vs. 6 patients). 24 women gave birth to 39 children. Mean age at first pregnancy was 28 years (range 19-42). No mortality during
pregnancy was seen. QoL measured with EQ5D-scale was affected by additional diagnoses (p=0,02), symptoms (p=0,01), warfarin
treatment (p=0,01) and surgery (p=0,02).
Conclusion: Women with Marfan were older than men. Pregnancies among women with Marfan are associated with increased risk
of aortic dissection, but no mortality during pregnancy was found in this study. Surgery, additional diagnoses, symptoms and
warfarin treatment were associated with lower QoL. F/U intervals were long with average less than one visit every two years. The
number of valve surgeries was high compared to aortic graft only, indicating late intervention. With shorter interval between aortic
examinations more valve sparing operations could be anticipated avoiding warfarin treatment.
11. The Effect of Folic Acid Fortification on the Birth Prevalence of Severe Congenital Heart Disease.
Ionescu-Ittu R, Marelli AJ, Mackie AS, Pilote L- McGill Adult Unit for Congenital Heart Disease, Montreal, QC, Canada
Background: Preconception intake of folic acid reduces the risk of neural tube defects and may have a similar protective effect on
congenital heart defects (CHD). Basic science evidence suggests that there is a possible protective mechanism, especially for
conotruncal heart defects. We conducted a population-based study to compare birth prevalence of severe heart defects before and
after the implementation of the mandatory fortification of flour and pasta products in Canada in 1998.
Methods: The study population consisted of live and stillbirths identified with severe heart defects (tetralogy of Fallot, endocardial
cushion defects, univentricular hearts, truncus arteriosus or transposition complexes) from 1990 to 2004 in Quebec administrative
databases. Patients were further grouped into patients with severe conotruncal defects (tetralogy of Fallot, truncus arteriosus and
transposition complexes) and patients without any severe conotruncal defect. Data was analyzed in two time periods: before-
fortification (1990-1998) and after-fortification (1999-2004). Birth prevalence was measured yearly as births (live and still births)
with severe heart defects per 1,000 births in Quebec. Poisson regression was used to estimate time trends in the birth prevalence of
severe defects in the period before and after fortification. We used the interaction between the period (before/after fortification) and
calendar time to test the significance of the change in time trend between the two periods.
Results: We identified 1,947 births with severe CHD over the 14-year study period, corresponding to an average birth prevalence of
1.56 per 1,000 births. Of these, 1,153 (59%) had a severe conotruncal defect (average birth prevalence 0.92 / 1,000 births). Time
trend analysis has shown that there was no change in the birth prevalence of severe birth defects in the period before fortification
(RR 1.01, 95% CI: 0.99-1.04), while in the period after fortification there was a statistically significant 5% decrease per year (RR
0.95, 0.91-1.00). The change in time trend between the two periods was statistically significant (p-value interaction = 0.01).
Subgroup analysis revealed similar time trends: for conotruncal defects we observed a slight increase in birth prevalence in the
period before fortification (RR 1.03, 1.00-1.06) that was curved in the period after fortification (RR 0.96, 0.91-1.02), while for non-
conotruncal neither trend reached statistical significance. The change in time trend between the two periods was statistically
significant for conotruncal defects (p-value interaction = 0.04), but not for non-conotruncal defects (p = 0.16).
Conclusion: Public health measures to increase folic acid intake were followed by a decrease in the birth prevalence of severe heart
defects, supporting the hypothesis that folic acid has a preventive effect on heart defects. The protective effect seemed stronger for
conotruncal defect, consistent with the biologic mechanism suggested in basic science studies.
12. An important step in the discovery of genes implied in cardiac development: autosomal dominant left atrial isomerism with
suggestive linkage to chromosome 9.
Van Engelen K, Van de Meerakker JBA, Lam J, Lekanne dit Deprez RH, Mathijssen I, Wilde AAM, Baars, MJH, Mulder BJM,
Moorman AF, Postma AV- Academic Medical Centre, Amsterdam, Netherlands
Background: Left atrial isomerism is a laterality disorder, which is characterized by bilateral left sidedness of the heart, commonly
associated with bilateral bilobed lungs and polysplenia.
It results from failure of genetic and embryonic pathways to establish normal left-right asymmetry, leading to a wide spectrum of
cardiac anomalies. The genes that are involved in cardiac development and the establishment of normal left-right asymmetry are
only partially known. We investigated a large three generation family presenting with left atrial isomerism to identify the underlying
genetic cause and thereby gain further insight in genes implied in laterality disorders and the development of the normal left-right
Methods: We obtained phenotypic information, including physical examination, electro- and echocardiography, and blood samples
of 19 family members. Genome wide linkage analysis and sequence analysis of candidate genes were performed subsequently.
Results: Thirteen of 19 family members had a cardiac anomaly. Some family members showed cardiac anomalies typically
associated with left atrial isomerism, such as monoatrium with bilateral left atrial appendages or atrioventricular septal defect with
persistent left superior caval vein. Other cardiac anomalies were present in some family members as well, e.g. tetralogy of Fallot and
atrial septal defect. In several asymptomatic individuals, electrocardiography revealed a typical configuration of low atrial rhythm
compatible with absent sinoatrial node. The condition was inherited in an autosomal dominant pattern. Genome wide linkage
analysis demonstrated linkage to a single locus on the long arm of chromosome 9 shared by all affected individuals, with a
multipoint maximum LOD score of 2.20 at marker D9S283. The shared locus was delineated by markers D9S167 and D9S1677 is
26 Mb in size and currently contains 218 genes. Sequence analysis of three candidate genes in this region (Inversin, TGFBR1 and
IPPK) revealed no mutations.
Conclusion: We identified a large family with autosomal dominant left atrial isomerism and mapped a suggestive locus for the
condition to chromosome 9. Identification of the underlying genetic defect will lead to a better understanding of cardiac
development and the etiology and pathogenesis of laterality disorders.
13. Hypervascular Hepatic Nodules in the Fontan Circulation: What do they mean?
Bryant T, Burney K, Stedman B, Vettukattill J, Haw M, Salmon A., Keeton B, Cope R, Hacking CN, Breen DJ, Sheron N, Millward-
Sadler H, Veldtman GR- Southampton General Hospital, Southampton, United Kingdom
Background: The Fontan circulation results in raised and often pulsatile central venous pressures, depressed cardiac output, and
variable cyanosis are known risk factors in the development of cardiac cirrhosis. Liver disease in patients with a Fontan circulation
has become more important as survival improves. Hepatic imaging in these patients has identified the presence of intrahepatic
arterialized nodules not previously described. We attempt to correlate the presence of these nodules with the underlying
Methods: Data on 27 Fontan patients (21 atrio-pulmonary, 3 total cavo-pulmonary, 2 right atrial (RA) - right ventricular and 1
Kawashima variant; mean age 25.2±5.7yr) investigated between September 2003 - January 2007 were reviewed. The presence of
arterialized nodules was examined in relation to other imaging findings including varices, portal venous phase hepatic enhancement
characteristics, (normal, reticular and zonal) and portal vein dimensions in addition to candidate hemodynamic variables (RA
pressure, RA saturation, aortic saturation).
Results: Reticular and zonal enhancement was a continuum with no significant hemodynamic correlation. Patients (7 of 27) with
arterialized nodules (size 0.5-3.2cm) had higher mean RA pressures (17.7±5.6SD vs. 13.1±4SDmmHg, P=0.025), where as their
mixed venous saturation and aortic saturation was not significantly different (70 %( ±11.9SD) vs. 66.5 %( ±9.13SD) and 91.7 %(
±9.5SD) vs. 94.1 %( ±4.3SD)).
Splenic size was abnormal in 9 (mean size 15cm, range 13-20cm), but no correlation with nodules was seen. Of 7 patients with
extra-hepatic varices, 2 also had lienorenal shunts. Patients with varices had higher mean RA pressures, (16.9±6.7SD vs.
13.4±3.8SD mmHg, p=0.0499) and higher RA saturations, (75±5.3SD vs. 65.5%±9.7SD, p=0.016). Again no correlation with
nodule formation was noted.
Conclusion: The Fontan circulation is almost uniformly associated with an altered liver perfusion as detected by a reticular or zonal
perfusion pattern in the portal venous phase during CT imaging of the abdomen. This appearance is similar to that seen in various
other chronic venous outflow conditions. Arterialized nodules commonly arise in this setting, usually peripherally in areas of most
pronounced fibrosis radiologically. Our hypothesis is that these nodules represent arterialization of the liver secondary to deprivation
of portal vein inflow due to raised central pressures. This is supported by a recent histopathological study of several nodules
suggesting that they are consistent with focal nodular hyperplasia on a background of hepatic vein centered liver fibrosis.
14. Superior Venous Baffle Stenosis Following an Atrial Switch Procedure: An Under-Recognized Problem.
Bottega N, Silversides CK, Oechslin EN, Dissanayake K, Harrison JL, Harris L- Adult Congenital Heart Disease Program, Peter
Munk Cardiac Centre, Toronto General Hospital, University of Toronto, Toronto, ON, Canada
Background: Patients with atrioventricular concordance, ventriculoarterial discordance (DTGA) and an atrial switch (Mustard or
Senning) procedure (ASP) may develop baffle stenosis. We evaluated the frequency of superior venous baffle (SVB) stenosis in an
adult cohort of these patients.
Methods: From the database of the Toronto Congenital Cardiac Centre, we identified all living patients >18 years of age with
DTGA who had an ASP and a cardiac imaging study (cardiac computed tomography, cardiac magnetic resonance imaging,
venography, or cardiac catheterization) after 2001 or before implantation of a transvenous pacemaker (PM) or implantable
cardioverter defibrillator (ICD). SVB narrowing was defined as a transverse luminal diameter <10 mm. Hemodynamically
significant narrowing was defined as at least one of: 1) azygous vein dilatation with retrograde flow, 2) extensive venous
collateralization, 3) clinical suggestion of superior vena caval syndrome, or 4) need for intervention on the SVB.
Results: One hundred and forty-two patients with ASP were identified from the database. Data were not available for 18 and 4
patients with and without a PM or ICD respectively due to the lack of, or inability to interpret, available SVB imaging. In total, 120
patients (79 males) age 31  6 years (range 21-49) were included. There were 29 (24%) and 6 (5%) patients with a PM or
ICD wire traversing the SVB respectively. SVB narrowing was observed in 45/120 (38%) patients and hemodynamically significant
narrowing in 13 of the 45 (29%). The mean SVB size was 7.36  2.67 mm (range 3-14) X 11.10  4.92 mm (range
3-21) in this group. Symptoms consistent with superior vena caval syndrome (facial fullness, headache and chest discomfort) were
described by 4 of the13 patients. Amongst the 17 patients with a PM or ICD with adequate cardiac imaging, 14 had SVB narrowing
which was hemodynamically significant in 8 patients. Three patients required dilatation : stenting for SVB narrowing
before device implantation.
Conclusion: Following ASP, approximately one third of adult patients have evidence of SVB narrowing on cardiac imaging and of
these, hemodynamically significant narrowing is present in almost one third. The implantation of a PM or ICD may further
predispose to baffle stenosis in patients with occult SVB narrowing and/or a predisposition to SVB stenosis. SVB patency should be
evaluated in all ASP patients prior to device implantation. Dilatation and stenting of a narrowed SVB should be considered prior to
transvenous lead implantation.
15. Management of High Risk Cardiac Patients in Pregnancy – Experience from a Single UK Centre.
Bowater S, Mensforth S, Hudsmith C, Thompson P, Thorne S- Queen Elizabeth Hospital Birmingham, Birmingham, United
Background: Heart disease is the leading indirect cause of maternal death in the UK and a significant cause of maternal morbidity.
In modern practice congenital heart disease accounts for many of the patients seen, and as survival from surgery continues to
improve we will see an increasing number of women with palliated complex congenital heart disease reach child bearing age. We
run a Joint Cardiac and Obstetric clinic, serving a population of 3 million women. We see patients with known heart disease for pre-
pregnancy counseling and risk stratification; manage high-risk patients during their pregnancy and also lower risk patients from the
local catchment area. This study looks at our management of patients with high-risk heart disease.
Methods: We retrospectively reviewed the Cardiac-Obstetric database for all patients with 2nd and 3rd trimester pregnancies seen
in the Cardiac Obstetric joint clinic between 2002 and 2007. 1st trimester miscarriages were excluded due to a lack of complete data.
Patient risk was determined by a modified ESC hierarchal model.
Results: 208 patients with 312 pregnancies were seen. Of these 101 pregnancies (71 women) were classified as high risk including
10 pregnancies in patients with a Fontan circulation, 31 with systemic right ventricles, 16 with at least moderate left sided
obstructive lesions, 2 with severe cyanosis and 6 with prosthetic valves. 1 patient had a termination on medical grounds (severe
subaortic stenosis), 4 patients had 2nd trimester miscarriages and 10 are currently pregnant thus we have information on 86
completed pregnancies. 38 (44%) pregnancies resulted in premature delivery (<37 weeks), gestation range 25-41 weeks. There were
2 neonatal deaths, 9 deliveries were complicated by fetal distress and 1 case of fetal heart block. Maternal complications included 4
women admitted with heart failure requiring intravenous diuretics, 1 woman requiring mitral valvuloplasty during pregnancy for
severe mitral stenosis (MS), 1 case of pulmonary edema post delivery in a patient with severe MS, 1 case of mechanical aortic valve
thrombosis requiring emergency caesarean section followed by valve replacement, 2 women required DC cardioversion during
pregnancy for atrial tachycardia (one Fontan and one mechanical mitral valve), another Fontan patient developed VT requiring
amiodarone and 1 patient with a previously unknown DCM developed severe heart failure requiring prolonged hospitalization.
There was 1 late maternal death 6 months following early post natal homograft AVR for severe aortic stenosis.
Conclusion: This study demonstrates that our practice of managing high risk patients in a joint Cardiac-Obstetric clinic allows
successful pregnancies in the majority of these patients with 95% of pregnancies resulting in a live birth and a maternal mortality of
1%. Complications may still arise, most notably prematurity, emphasizing the need for care in a specialist unit with a specialist
multidisciplinary team including Cardiologist, Obstetrician, Anesthetist and Midwives for optimal antenatal, perinatal and postnatal
care. We recommend pre-pregnancy counseling in all women with heart disease to provide adequate information on potential risks
enabling the woman make an informed decision. This study has looked retrospectively at events during the pregnancy and late
deaths attributed to the pregnancy. We are currently prospectively investigating the late effects of pregnancy on this high-risk group.
16. Mechanical Valve Prosthesis in the Pulmonary Position: A Single Center Experience in Grown-Up Congenital Heart
Dos L, Muñoz C, Mendez A, Ginel A, Montiel J, Padrol D, Padro JM, Subirana M - Hospital de la Santa Creu i Sant Pau, Barcelona,
Background: Pulmonary valve replacement is becoming increasingly frequent in the cardiac surgery departments due to the
development of long term complications in the growing population of adults with repaired congenital heart defects. The selection of
the type of prosthesis is controversial: bioprosthesis have a more widespread use but have limited longevity and, on the other hand,
mechanical prosthesis in the right heart seems to be at high risk of thrombosis. The objective of the study was to assess the outcome
of the mechanical valve prosthesis in the pulmonary position implanted in our institution in the population of grown-up congenital
heart disease, with special attention to thrombotic and bleeding complications.
Methods: From 1977 to 2007, 22 consecutive grown-up congenital heart disease patients underwent a total of 24 pulmonary valve
replacements with mechanical prosthesis in a single tertiary referral center. Preoperative, intraoperative and follow-up information
were obtained from chart review. They were predominantly male (n=18, 75%) and the most frequent underlying cardiac condition
was tetralogy of Fallot (n=15, 62%) with a mean age at the time of pulmonary valve replacement of 32,6±10,5 years (range 16,08-
49,88 years). Bileaflet prosthesis were used in the 67% of the cases (n=16) and tilting disc prosthesis in the remaining 33% (n=8).
Patients were discharged on oral anticoagulation to maintain an international normalized ratio of 2.5-3.5.
Results: The postoperative mortality rate was 4,2% (n=1) with no late deaths documented after a mean follow-up of 6,92±7,45
years (range 0,18-24 years). No major bleeding episodes occurred. A total of 8 episodes of valve thrombosis despite correct
anticoagulation were documented. All episodes occurred in two patients (3 and 5 thrombosis respectively) with bileaflet prosthesis
and severe right ventricular dysfunction. Thrombolysis was successful in all the cases and, after addition of antiplatelet treatment to
the oral anticoagulation, no further episodes of thrombosis were documented.
Conclusion: In our experience, mechanical valve prosthesis may be an alternative to bioprosthesis in grown-up congenital heart
disease patients undergoing pulmonary valve replacement. However, some patients are prone to develop recurrent valve thrombosis,
probably those with less favorable hemodynamics (bileaflet prosthesis and severe right ventricular dysfunction). Addition of
antiplatelet treatment to optimal oral anticoagulation may prevent valve thrombosis in these high-risk patients.
17. Outcome after Orthotopic Heart and Heart-Lung Transplantations in Adults with Congenital Heart Disease.
Le Gloan L, Landréat J, Guérin P, Gournay V, Pattier S, Treilhaud M, Baron O, Trochu JN- Institut du Thorax, CHU Nantes,
Background: Because of the advances in paediatric cardiology and surgery, an increasing number of children with complex
congenital heart disease reaches adulthood. In some patients, heart or heart-lung transplantation is evoked because of severe
myocardial dysfunction, with all anatomic and physiologic challenges implied.
The aim of our work was to study the survival rates of heart and heart-lung transplants in adults with congenital heart disease
Methods: Demographic, intra- and postoperative data in all ACHD who underwent a heart or heart-lung transplantation between
1985 and 2007 at the teaching Hospital of Nantes, France, were reviewed. Premature and late mortality were evaluated with Kaplan-
Meier survival estimates. A case-control study was added for both groups of transplantation, matching each congenital transplanted
patient, to a transplanted patient for acquired heart disease.
Results: Within this period, 564 patients underwent a cardiac transplantation, including 11 for congenital heart disease (26 ± 10
years), with a follow up of 4.5 years. Double discordance was the most frequent pre-transplant diagnosis (3 patients). Two patients
died early after heart transplantation. Previous cardiac surgery was more frequent in patients transplanted for congenital cardiac
reasons compared to the matched transplanted patients. Survival at 1 month, 1 year and 5 years was 82 % in each case, and no
significant differences were observed when compared to the matched transplanted patients. 100 patients underwent a heart-lung
transplantation, including 12 for congenital heart disease (31 ± 9 years). They were followed during 6.5 years. Five patients died,
and among them 3 early after heart-lung transplantation. Survival at 1 month, 1 year and 5 years was respectively 82%, 64% and
53% without significant differences in comparison to the non-congenital matched transplanted patients.
Conclusion: Despite a high early mortality, the overall survival rates after heart and heart-lung transplantations in adults with
congenital heart disease observed in this study, are encouraging, with survival rates similar to those of adults transplanted without
congenital heart disease. However, these results may evolve in the following years, as the number of high-risk patients on waiting
lists increases, due to improvements in medical and surgical care of ACHD.
18. Use of Tricuspid Annular Motion to Quantify Right Ventricular Systolic Function in Repaired Tetralogy of Fallot:
Traditional and Novel Echocardiographic Techniques Compared with Ejection Fraction by Cardiac Magnetic Resonance
Lyseggen E, Wald R, Redington AN, Silversides CK, Siu SC, Provost Y, Oechslin EN- Adult Congenital Heart Disease Program,
Peter Munk Cardiac Centre, Toronto General Hospital, University of Toronto, Toronto, ON, Canada, and, Department of
Cardiology, University of Oslo, Rikshospitalet, Oslo, Norway
Background: Tricuspid annular plane systolic excursion (TAPSE) and other measures of tricuspid annular motion have been
demonstrated to accurately reflect right ventricular (RV) ejection fraction (EF) and prognosis in various patient populations,
however, the usefulness of these parameters in assessment of RV function in adults with repaired tetralogy of Fallot (TOF) has not
been specifically studied. We aimed to compare assessment of tricuspid annular motion by M-mode (TAPSE), tissue Doppler
echocardiography (TDE) [displacement, peak ejection velocity, isovolumic myocardial acceleration (IVA)] and speckle tracking
echocardiography (STE) [displacement] with RVEF obtained by cardiac magnetic resonance (CMR).
Methods: We prospectively recruited 34 consecutive patients (mean age 35±12 years) with repaired TOF (age at repair 8±7 years).
Echocardiography and CMR imaging were performed on the same day. TAPSE was measured from systolic displacement of the RV
free wall/tricuspid annular plane junction by M-mode. For all other echocardiographic parameters the sample volume was placed in
the basal RV free wall. RVEF was calculated by CMR on a commercially available scanner using validated software.
M-mode and TDE parameters were recorded as an average of 3 consecutive cycles. For STE, 1 cycle was used for analysis.
Results: There were correlations between RVEF calculated by CMR and the following echocardiographic parameters: TAPSE
(r=0.53, y=0.28x+5.84, P=0.001), displacement by TDE (r=0.43, y=0.19x+3.99, P=0.01) and displacement by STE (r=0.39,
y=0.23x+7.29, P=0.02). There were no statistically significant correlations between RVEF and either peak ejection velocity (r=0.33,
P=NS) or IVA (r=0.03, P=NS).
Conclusion: TAPSE by traditional M-mode demonstrates better correlation with RVEF as compared with TDI and STE measures
for assessment of tricuspid annular motion in TOF. However, regardless of these differences, none of the echocardiographic
measures of tricuspid annular motion are reliable measures of RVEF in patients with repaired TOF. We speculate that 1) systolic
dysfunction/pathologies of the RVOT in these patients may be poorly reflected by TAPSE and 2) the novel echocardiographic
techniques may be more susceptible to poor image quality.
19. Detailed description of cardiovascular Findings in Loeys-Dietz Syndrome – An aggressive aortic aneurysm syndrome with
widespread vascular involvement.
De Backer J, Loeys B, Spevak P, Dietz H- University Hospital Ghent, Belgium
Background: Loeys-Dietz syndrome (LDS) is a novel aortic aneurysm syndrome characterized by the triad of hypertelorism, bifid
uvula and/or cleft palate, and aortic aneurysm/dissection with generalized arterial tortuosity.
LDS is caused by mutations in the genes encoding either of the transforming growth factor receptors (TGFBR1 or 2). Life
expectancy is reduced and mainly determined by cardiovascular complications.
Objective: To provide a detailed description of cardiovascular findings in LDS patients.
Methods: We studied 47 subjects (mean age 18,8yrs, 47% males) with clinical manifestations and/or a positive family history of
LDS and the presence of a TGFBR1 or 2 mutation. For the assessment of cardiovascular involvement, all 47 subjects underwent
echocardiography and CT or MRI of the chest; 34 patients also underwent CT or MRI of the abdomen and 36 patients had CT/MRI
of the brain. Serial images were performed in 10 patients.
Results: The most common cardiovascular finding in these patients was aortic root aneurysm (89%), but aneurysms at the
descending thoracic or abdominal aorta (27,3 and 26,5% respectively) and tortuosity or aneurysms of the major thoracic and
abdominal branching vessels (43%) and head and neck vessels (73,3%) were also frequent. Main pulmonary artery dilatation was
encountered in 24 patients (56%). Six patients had a patent ductus arteriosus. Mild LV dilatation occurred in 12 patients (27,3%). 75
% of patients had three or more cardiovascular lesions.
The age range at which the lesions were detected varied widely (in utero- 37yrs). Ascending aortic aneurysms evolved rapidly with
an annual increase exceeding 5%
Conclusion: In this large series of LDS patients, we documented a widespread vascular involvement including: the aorta, major
branching vessels and main pulmonary artery. There is evidence for mild LV dilatation. Medical diagnosis and follow-up should
include imaging of the entire vascular tree and echocardiography at regular intervals.
20. The Cardiac Adaptation to Pregnancy is Impaired in Women with Congenital Heart Disease.
Karamermer Y, Van Geuns RJ, Cornette JMJ, Moelker A, Kirschbaum SW, Johnson M, Pieper PG, Steegers EA, Roos-Hesselink JW
- Department of Cardiology, Erasmus MC, Rotterdam, Netherlands
Background: Pregnancy is a major issue in the management of women with Congenital Heart Disease (CHD). Complications such
as growth retardation, premature birth and perinatal mortality are more frequent in their offspring. The pathophysiology of these
complications may be related to a diminished ability to increase Cardiac Output (CO). Normally, CO increases with 40-50% during
pregnancy. Patients with CHD were investigated outside and during pregnancy with cardiac magnetic resonance imaging (cMRI).
Methods: Eight women with CHD (2 aortic valve replacement, 2 Fallot, Ebstein, ventricular septal defect, atrioventricular septal
defect, pulmonary stenosis) were evaluated with cMRI at 20 weeks and 32 week of gestation and compared with measurements six
to twelve months preconceptionally (n:3) or postpartum (n:5).
Results: CO decreased significantly from 6.9 L/min at 20 weeks to 5.4 L/min at 32 weeks of gestation (p: 0.02). In addition a
significant reduction in end-diastolic volume and stroke volume between 20 and 32 weeks of gestation was observed (resp. 146.3 vs.
127.9 ml and 77.4 vs. 62.0 ml), with a decline in ejection fraction from 53.4% to 48.9% (p: 0.05). There was a significant increase in
left ventricular mass from 87.5 gram at 20 weeks to 94.4 gram at 32 weeks of pregnancy.
Conclusion: Instead of an increase in CO between 20 and 32 weeks of gestation, we observed a significant fall in CO of 20%.
Although the number of subjects in this study is limited, this finding was observed in 7 of the 8 patients and might explain the
increased rate of complications during pregnancy in this population.
21. Single Ventricle vs. Double Ventricle in Unpalliated Cyanotic Adult Survivors: Is There an Advantage?
Broberg C, Weiss J, Heading J, Menashe V, Pantely G- Oregon Health and Science University, Portland, OR, United States
Background: Theoretically two ventricles ought to be better than one. However, many long-term unpalliated survivors with a single
ventricle anecdotally do not appear to have a disadvantaged clinical course. We sought to compare the clinical status of cyanotic
patients with one vs. two ventricles.
Methods: Cyanotic patients with unpalliated single ventricle physiology were identified and compared to Eisenmenger VSD
patients. Data from ECG, MRI, 6-minute walk testing, and cardiopulmonary exercise were obtained retrospectively. Not all data
were available on all patients.
Results: Twelve single ventricle patients were identified (7 female, 5 male). Anatomic diagnoses were DILV in 6, TA in 4, DIRV in
1, and unbalanced AVSD in 1. Three patients had pulmonary stenosis, and 1 had a prior PA banding, although none were felt
suitable for surgical palliation. Compared to 19 double ventricle patients, age, gender distribution, and resting oxygen saturation
were similar. Resting heart rate was lower, and there was a trend toward a longer QRS duration. PR interval was not significantly
different, although this excluded one single ventricle patient with complete heart block. There was no difference in systemic
ventricular ejection fraction by MRI.
Exercise duration was greater in the single ventricle patients, but there was otherwise no difference in any exercise parameter
including 6-minute walk test difference, peak oxygen consumption, or percent-predicted oxygen consumption. Peak heart rate
obtained tended to be lower in the single ventricle group, reflecting the lower resting heart rate, but heart rate reserve was not
different. Oxygen saturation and exercise capacity did not differ between patients with and without pulmonary stenosis.
Conclusions: Despite the theoretical disadvantage of having one functional ventricle, unpalliated single ventricle patients are not
different functionally from other cyanotic patients with two ventricles. This implies that the single ventricle may be more capable
than otherwise believed, at least in these long-term survivors.
22. The Impact of Pregnancy on the Systemic Right Ventricle: Experience from a Single UK Centre.
Hudsmith L, Bowater SE, Clift PFJ, Thompson P, Thorne SA- The Queen Elizabeth Hospital, Birmingham, United Kingdom
Background: Right ventricular dilatation, dysfunction and arrhythmias are a significant cause of morbidity and mortality in patients
with a systemic right ventricle. Females who underwent an atrial switch (Mustard and Senning) surgical correction of Transposition
of the Great Arteries are currently undertaking pregnancies. Little is known about the impact of pregnancy and its hemodynamic
effects on the systemic right ventricle and there are concerns about the potential deterioration of these patients in the short and long-
term. We aimed to investigate the effects of pregnancy on patients with surgically corrected and physiologically (ccTGA) systemic
Methods: Notes of 17 females with 26 completed pregnancies and a systemic right ventricle between 1999 and 2007 were
retrospectively analysed. Information regarding clinical status, echocardiographic evaluation before and after pregnancy, medication
and cardiac, obstetric and neonatal complications were recorded.
Results: 26 pregnancies were completed in 17 females (11 Mustard, 10 Senning, 5 ccTGA) at a mean age of 26 ± 6 years.
Echocardiographic data on RV function was available on 20 patients and systemic AV valve regurgitation on 22. Prior to pregnancy,
RV function was good in 5, mildly impaired in 12, moderately impaired in 2, severely impaired in 2. There was deterioration in
systemic right ventricular function in 25% (5) of patients following pregnancy. Systemic AV valve regurgitation was absent in 2
women, mild in 13, moderate in 7 before pregnancy, advancing in 3 (14%)women post-pregnancy. There was also a significant
increase in NYHA class following pregnancy, p<0.01.
NYHA class was I in 20 and II in 1 pre-pregnancy and I (13) and II (8) following pregnancy (5 data not available).
Delivery details were available on 20 pregnancies: 4 spontaneous vaginal delivery, 9 emergency caesarean section, 3 elective
caesarean section, 4 assisted delivery (3 ventouse and 1 forceps). Mean gestation was 36 ± 3 weeks (range 30-40), mean weight
2350 ± 665g, with 45% premature delivery (<37 weeks). There was 1 neonatal death from sepsis, 1 fetal heart block, 2 episodes of
fetal respiratory distress and no recurrence of congenital heart disease. There were no maternal deaths. 2 patients suffered significant
arrhythmias in pregnancy: 1 supraventricular tachycardia and 1 with non-sustained ventricular tachycardia who underwent
successful ablation following delivery.
Conclusion: Pregnancy in patients with a systemic right ventricle is clinically well-tolerated but is associated with an increase in
NYHA class, deterioration in right ventricular function and increase in systemic AV valve regurgitation. Patients must be
appropriately counseled about these potential deleterious effects of pregnancy and must be followed carefully with close
collaboration between adult congenital cardiologists and obstetricians throughout and after pregnancy.
23. Men’s Health in Patients with Congenital Heart Disease.
Tanous D, Kovacs A, Llano M, Kulikowski J, Silversides CK, Oechslin EN- Adult Congenital Heart Disease Program, Peter Munk
Cardiac Centre, Toronto General Hospital, University of Toronto, Toronto, ON, Canada
Background: Erectile dysfunction (ED) frequently occurs in men with coronary artery disease and chronic heart failure. However,
not much is known about ED in adults with congenital heart disease (CHD). The purpose of this study is to determine the
prevalence and associated factors of ED in men with CHD.
Methods: Adult males with CHD attending outpatient clinics at a single centre were approached to complete the following
questionnaires: a) International Index of Erectile Function (IIEF-15) questionnaire, which includes items corresponding to the
Sexual Health Inventory for Men (SHIM); b) International Prostate Symptom Score (I-PSS), which is an assessment of the presence
and severity of lower urinary tract symptoms/benign prostatic hypertrophy; c) Short Form-12 (SF-12), used as a measure of
perceived physical and mental health status and d) Satisfaction with Life Survey, a 5-item measure of global life satisfaction.
Correlational analyses and independent sample t-tests were performed in order to determine whether symptoms of erectile
dysfunction differed as a function of age, cardiac defect complexity, NYHA class or whether they were taking medications with
Similar statistical analyses were conducted in order to determine whether the following differed as a function of the presence or
absence of erectile dysfunction: lower urinary tract symptoms, perceived health status and satisfaction with life.
Results: Of 82 respondents, 68 were in stable relationships or sexually active and were therefore suitable for analysis of erectile
dysfunction. The average age of respondents was 35 ± 12 years. Twenty-nine percent (19/66) reported at least some degree of ED,
of whom 7 reported severe ED symptoms. Compared to patients without ED, patients with ED had significantly poorer perceived
physical and mental health (p < 0.05) and significantly lower life satisfaction (p <0.01). Worsening ED was also significantly
correlated with increasing age (p < 0.05) and more severe lower urinary tract symptoms (p < 0.05). Compared to patients in NYHA
Class I (n = 45), patients in Class II-III (n = 21) did not have significantly more severe ED symptoms (p = 0.2). There was no
significant difference in the extent of ED symptoms between patients with cardiac defects of simple or moderate complexity (n =
33) versus those with defects of great complexity (n=33, p = 0.6) or patients who were (n = 28) and were not (n = 38) taking
medications with antihypertensive properties (p = 0.6).
Conclusion: In this group of adult males with congenital heart disease, there was a high incidence of erectile dysfunction.
Increasing age, worsening lower urinary tract symptoms, poorer perceived mental and physical health and lower global satisfaction
with life were associated with the presence or extent of ED. Erectile dysfunction, however, did not differ according to the
complexity of congenital heart disease or functional status. Vigilance and sensitivity to men’s health issues must be recognized
during our daily practice, even in men with CHD of simple and moderate complexity.
24. Adult congenital heart surgery: Children’s hospital or adult hospital?
Kogon B, Plattner C, Kirshbom P, Kanter K, Lyle T, Jennings S, McConnell M, Book W- Emory University Children's Healthcare of
Atlanta, GA, United States
Background: One of the current controversies in the field of adult congenital heart disease is whether patients should be cared for at
an adult hospital or a pediatric hospital. Our program is unique in that we have experience with both systems. Over the last 7 years
we have slowly transitioned our program from the children’s hospital to the adult hospital. We analyzed our experience with each
Methods: Between 2000 and 2007, 303 operations were performed on adults with congenital heart disease. Medical records were
reviewed. 185 operations were performed at the adult hospital and 118 were performed at the children’s hospital. Forty-six
operations were performed by an adult heart surgeon and 257 by a congenital heart surgeon.
Initial congenital diagnoses were categorized: complex (147), congenital valve disease (65), septal defects (64), thoracic vascular
(8), arrhythmias/dysrhythmias (10), and other (9). Operations were categorized by primary procedure: valve repair/replacement
(179), complex open procedure (17), septal defect (39), thoracic vascular (8), pacemaker/AICD (44), heart failure (10), and other
Results: Throughout the transition, the number of cases performed annually at the adult hospital gradually increased from 7% to
78%. Ages ranged from 18 to 75 years (median 27) and were significantly higher in the adult hospital group (median 34 vs. 20
years). There was a difference in the distribution of procedures among the hospitals, with a predominance of pacemaker/AICD
procedures performed at the children’s hospital and valve repair/replacement procedures at the adult hospital. Coexisting medical
problems were present in 45% patients and were significantly more common in the adult hospital group (62 vs 18%). Mortality was
14/303 (4.6%). Risk factors for mortality included older age at the time of surgery (p=0.021), surgery performed at a children’s
hospital (p=0.012), and surgery performed by an adult heart surgeon (p=0.001).
Conclusion: Congenital heart surgery can be performed in adults with reasonable morbidity and mortality. When surgery becomes
necessary, these adult patients may be best served by a congenital heart surgeon. Also, caring for an anticipated aging adult
congenital population with increasingly numerous coexisting medical problems may be facilitated in an adult hospital setting.
25. 61 Contegras in adults: Results at 4.5 years after implantation for right ventricular outflow tract reconstruction in patients
with congenital cardiac malformations.
Boethig D, Westhoff-Bleck M, Ono M, Goerler H, Breymann T- Hannover Medical School, Germany
Background: Reconstruction of the right ventricular outflow tract (RVOT) in adults is often performed using homografts. They
have acceptable long term results, but they are not ideal, so alternatives deserve to be evaluated. The Contegra (Medtronic Inc,
Minneapolis, MN, USA), a bovine jugular vein segment with a naturally grown integrated valve, has proven to compare well to
homografts implanted in pediatric RVOTs. In contrast to homografts, the jugular veins are easier available and easier to implant.
Here we describe the results obtained in adult patients with this device.
Methods: Between July 2003 and February 2008, 61 Contegra conduits were implanted in our institution in the pulmonary position
for RVOT reconstruction in 59 adult patients. 29 (47.5%) were men, 32 (52.5%) women. Their age at implantation ranged from 18
to 65 years, mean 26 +- 8.6, median 23.6 years. The underlying congenital malformations were: tetralogy of Fallot (n=47, 77.0%),
pulmonary stenosis (n=5; 8.2%), pulmonary atresia (n=3; 4.9%), double outlet right ventricle (n=3; 4.9%) and 3 different other
malformations, including one Ross operation.54 patients (88.6%) had previous intracardiac repairs; 6 pulmonary artery conduits
were replaced (3 homografts, 2 Contegras and 1 valveless tube). 1 patient had not been operated before. Within a structured follow-
up program, we asked our patients and their cardiologists at least yearly for their functional echo- or angiocardiographic data and
eventual invasive procedures. More than half of them are being followed in the Hannover Medical School. Follow- up is complete.
Total follow-up time is 159 years, mean 2.6, range 0 - 4.5 years. Data were collected using FileMaker 8.5 (Filemaker Inc., Santa
Clara, CA, USA) and evaluated with SPSS V.15 (SPSS Inc, Chicago, IL, USA). Time dependent event rates were calculated
according to the Kaplan-Meier method and are given +- standard error.
Results: Survival at 4.5 years is 98.3 +- 0.17%; one patient died postoperatively (not conduit related, early death rate 1.7%), all
others are alive. Freedom from explantation at 4.5 years is 89.6 +- 5.0% (3 explants were due to endocarditis - at 2.4, 2.6 and 3
years, each after inadequate prophylaxis. 1 explant was required for degeneration after 1 year, resulting in severe insufficiency).
Freedom from endocarditis at 4.5 years is 91.4 +- 4.8%. 2 dilatations of stenoses at the distal anastomosis (at 2.0 and 2.6 years)
result in 94.6 +- 3.7% freedom from intervention at 4.5 years. These results are comparable to published homograft results in similar
Conclusion: At 4.5 years, the Contegra bovine jugular vein graft seems an alternative to homografts for the reconstruction of the
right ventricular outflow tract in adults.
26. Long Term Outcome of Repaired Tetralogy of Fallot. Experience of A National Adult Congenital Heart Disease Center in
Gonzalez R, Muñoz C, Uriarte P, Goset C- Instituto Nacional del Torax, Santiago, Region Metropolitana, Chile
Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD). In Chile, the first successful
congenital heart surgery with extracorporeal circulation was done in 1957. Since the year 2000, our institution has become the
national referral center for adults with CHD. The purpose of this communication is to present the long-term outcome of patients
after surgical repair of TOF and other diseases of similar pathophysiology, followed at our institution.
Methods: 125 patients, 73 male (58%), had surgical repair at a mean age of 10 : 9.6 years (range 0.5 to 49 years, median 7
years) in the period 1965 to 2006.
Their diagnoses were: TOF = 103 (82%), VSD with pulmonary atresia and confluent pulmonary arteries = 15 (12%) and DORV
with subaortic VSD and pulmonary stenosis = 7 (6%). 52 (42%) had a previous palliative surgery (classical or modified Blalock-
Taussig shunt). The surgical procedure included 59 (47%) with transanular patch (TAP) and 16 (13%) with a right ventricle to
pulmonary artery (RV to PA) conduit.
Results: Mean follow up was 16.5 +- 8.2 years (range 41.9 to 1.3 years). During this period: a) Only one patient died (0.8%). He
had severe RV heart failure and suffered septic complications from a pneumonia. b) Freedom from reoperation was related to the
surgical procedure performed: 42/50 (84%) with no TAP, 32/59 (54%) with TAP and 5/16 (31%) with RV to PA conduit (Long
Rank test p < 0.01 TAP compared to conduit, p < 0.0001 no TAP compared to conduit and p < 0.0001 no TAP compared to TAP). A
second reoperation was necessary in 1/7 with no TAP, 3/27 with TAP and 5/11 with RV to PA conduit. In this last group 1 had a
third and 1 had a forth conduit replacement. c) The causes of reoperation were also related to the surgical procedure performed:
Surgery PI PS PI+PS TI RVSD AI PAsS Other
TAP 16 2 6 4 3 2 2 2
No TAP 0 4 2 1 2 0 1 1
VD-PA 0 7 2 2 0 0 4 1
P = pulmonary, T = tricuspid, A = aortic, I = insufficiency, S = stenosis, RVSD = residual ventricular septal defect, PAs =
pulmonary arteriesd) 122/124 (98%) are in NYHA FC I and II, with only 2 in FC III.
Conclusion: In this series of patients with TOF and diseases with similar pathophysiology, the long-term outcome after surgical
repair has been very good, with very low mortality and excellent functional capacity. The use of a transannular patch or a RV to PA
conduit for the surgical repair was associated with a higher incidence of reoperations.
27. Exercise-induced hypertension as a predictor for sustained hypertension in adult post-coarctectomy patients.
Mackaij GJ, Bouma BJ, Van Montfrans GA., Van den Born BJH, Groenink M, Vriend JW, Mulder BJM- Academical Medical
Centre Amsterdam, Netherlands
Background: Post-coarctectomy patients often suffer from late onset hypertension. The purpose was to investigate whether
exercise-induced hypertension is a predictor for developing sustained hypertension in adult post-coarctectomy patients.
Methods: We prospectively examined 40 adult post-coarctectomy patients, mean age 37 yrs, 25 males, without significant
restenosis. Ambulatory blood pressure measurement (ABPM) and treadmill exercise testing were performed at baseline in 2001 and
at 6 years follow up in 2007. Patients who showed mean daytime systolic blood pressure readings ≥ 140 mmHg or mean
daytime diastolic blood pressure readings ≥ 90 at ABPM or were on antihypertensive drugs were considered sustained
hypertensive. Patients normotensive at ABPM, with a maximal systolic blood pressure ≥ 200 mmHg at exercise testing were
considered to have exercise-induced hypertension.
Results: At baseline 14 patients had sustained hypertension, 5 had exercise-induced hypertension and 21 were normotensive. At 6
years follow up from the 5 patients with exercise-induced hypertension at baseline, 4 patients (80%) developed sustained
hypertension. Of the 20 patients who were normotensive at baseline 2 patients (10%) developed hypertension.
Conclusion: These results suggest that exercise-induced hypertension is a predictor for developing sustained hypertension in adult
28. Novel Right Ventricular CMR models in Ebstein's Anomaly.
Hudsmith L, Bowater SE, Guest PJ, Thorne SA, Steeds RP, Clift PFJ- The Queen Elizabeth Hospital, Birmingham, United Kingdom
Background: The management of patients with Ebstein’s anomaly and identification of the optimal timing for surgery is difficult.
There are few well-validated predictors of prognosis and surgical outcome in these patients. We investigated the role of
cardiovascular magnetic resonance (CMR) in patients with Ebstein’s anomaly, aiming to develop novel prognostic measures of right
ventricular (RV) function. We describe the use of CMR to model and predict the potential functional capacity of the right ventricle
following surgical intervention.
Methods: 12 adult patients (5 male, mean age 39 ± 13 years, 22-59 years), NYHA 1.8 ± 0.8 (range I-III), underwent
cardiopulmonary exercise testing and CMR (1.5T, Symphony, Siemens, Germany). CMR analysis of all volumetric data was
performed using ARGUS analysis (Siemens) by consensus of 2 experienced operators. Potential post-repair volumes were modeled
by measuring end-diastolic and end-systolic volumes contained within right ventricular muscle whether atrialized or within the true
ventricle. Left ventricular parameters were contoured in the usual manner. Longitudinal function was measured using triscupid
annular planar systolic excursion (TAPSE).
Results: Mean VO2 max of the patients was 29 ± 10 ml/kg/min (13-46), 84 ± 21% predicted, RER 1.01 ± 0.09. 5 patients had
previously documented atrial arrhythmias. Using CMR, mean left ventricular ejection fraction (LVEF) was 64 ± 14%; right
ventricular ejection fraction (RVEF) was 35 ± 17% (17-83), RV end-diastolic volume (RVEDV) 160 ± 73ml (42-291), RV End-
systolic volume (RVESV) 100 ± 50ml (28-180).Potential post-repair volumes for RVEDV were 129% larger, 329 ± 164ml (160-
744), and 46% larger for RVESV, 215 ± 151ml (96-587) demonstrating significant contraction of the atrialized area. CMR TAPSE
was 25 ± 8mm (13-42) showing good longitudinal function.
Conclusion: CMR provides a unique tool to objectively quantify RV function in patients with Ebstein’s anomaly as well as to
provide detailed anatomical information. It not only permits accurate assessment of true RV function but allows direct quantification
of atrialized ventricular volumes and function. Utilization of CMR derived parameters in Ebstein’s anomaly may allow the
development of novel predictors of prognosis and optimize timing of surgical intervention.
29. Hematological parameters before and after treatment with bosentan in Eisenmenger’s syndrome.
Jensen A, Iversen K, Vejlstrup N, Sondergaard L- Kardiologisk klinik B, Rigshospitalet, Denmark
Background: Patients with Eisenmenger’s syndrome are cyanotic due to high pulmonary vascular resistance causing pulmonary to
systemic shunting through a cardiac defect, most often a VSD. The kidneys respond to hypoxia by increasing the secretion of
erythropoietin. This increases red blood cell production (secondary erythrocytosis) resulting in increased blood viscosity. Patients
with Eisenmenger’s syndrome are at increased risk of micro embolism due to hyperviscosity, but repeated phlebotomy is not a
solution, since this in it self pose the potential hazard of iron deficiency, microcytosis, and an increased risk of cerebrovascular
events. Until recently treatment options for patients with Eisenmenger’s syndrome were limited. However, pulmonary vasodilatation
with bosentan increases pulmonary blood flow, and is now an approved treatment for Eisenmenger’s syndrome.
The aim of this study was to investigate whether treatment with bosentan, will affect the erythropoietin production and diminish the
Methods: 21 patients with Eisenmenger’s syndrome (14 females) were treated with bosentan 125 mg bid for three months. Before
and after 3 months of treatment the patients were examined with, right heart catheterization to measure pulmonary blood flow, blood
samples were drawn to measure hemoglobin, hematocrit, and erythropoietin. The patients had a 6 minutes walk test (MWT) to
measure arterial oxygen saturation (SAT) at rest and after exercise.
Results: After three months of treatment with bosentan there was a significant increase in pulmonary blood flow from median 2.6
(range 1.1 – 4.7) to 3.5 (range 1.7 – 6.9) L/min (0.02 > p > 0.01)). The 6 MWT did not show any significant change in SAT before
(median 80 (range 57 – 93) to 80 (range 69 – 92) percent) and after exercise (median 61 (range 36 – 80) to 61 (range 30 – 81)
percent). Blood samples did not show any significant reduction in hemoglobin (median 11.7 (range 7.7 – 14.4) to 11.1 (range 8.9 –
14.8) mmol/L), hematocrit (median 0.59 (range 0.39 – 0.74) to 0.53 (range 0.41 – 0.78) or erythropoietin (median 26 (range 9.6 –
2070) to 18.6 (range 2.8 – 2850) IU/L).
Conclusion: Even though patients with Eisenmenger’s syndrome treated with Bosentan achieved a significant improvement in
pulmonary blood flow, this did not change SAT at rest or during exercise. Erythropoietin and hematological parameters showed a
trend towards lower values, but the change was not significant; it is therefore unlikely that bosentan will reduce the risk of micro
embolism or diminish the need for closely monitored phlebotomy in patients with Eisenmenger’s syndrome.
30. Variability in Practice Patterns Amongst Adult Congenital Heart Disease Practitioners Caring for Patients with Significant
Pulmonary Regurgitation After Tetralogy of Fallot Repair.
Wald R, Lyseggen E, Oechslin EN, Webb GD, Silversides CK- Philadelphia Adult Congenital Heart Disease Centre, Philadelphia,
PA, United States, and Adult Congenital Heart Disease Program, Peter Munk Cardiac Centre, Toronto General Hospital, University
of Toronto, Toronto, ON, Canada
Background: Pulmonary valve replacement (PVR) for significant pulmonary regurgitation (PR) is the most frequent indication for
late re-operation after tetralogy of Fallot (TOF) repair. Timing of PVR in the asymptomatic patient with significant PR remains
unclear. Various studies suggest that right ventricular (RV) volumes derived from cardiac magnetic resonance imaging (CMR) can
be helpful in determining timing of intervention. We sought to examine thresholds for PVR amongst adult congenital heart disease
(ACHD) practitioners caring for the asymptomatic patient with TOF.
Methods: Physicians attending a large, international ACHD conference were asked to complete a survey exploring practice patterns
of practitioners caring for adults with TOF, with particular focus on decision-making surrounding referral for PVR. All survey
responses were anonymized.
Results: A total of 128 physicians participated in the survey. Background training was pediatric cardiology 49%, adult cardiology
38%, combined adult/pediatric cardiology 9% and cardiovascular surgery 4%. The majority practiced in North America (56% in the
United States and 12% in Canada). Most respondents practiced in a tertiary care setting (84%), had cardiac surgery at their
institution of practice (86%) and routinely used CMR for TOF follow-up (72%).
The majority had >5 years of experience in ACHD (74%) and saw >5 TOF patients per month (70%). Given a scenario of an
asymptomatic TOF patient with significant PR and RV dilation, practice patterns amongst respondents varied. Nine percent felt that
there was no role for surgery in the asymptomatic TOF patient with normal RV ejection fraction (EF) and 4% felt that surgery was
not indicated despite RV dysfunction. Of those who felt that surgery was indicated, fewer practitioners relied on RV volumes as
RVEF decreased (26% did not use CMR volume thresholds if RVEF was normal, 28% did not use CMR volume thresholds with
mildly decreased RVEF [EF 45%], and 35% did not use CMR volume thresholds with moderately decreased RVEF [EF 30%]. For
those that used CMR RV volumes in surgical decision-making, RV end-diastolic volume thresholds varied depending on the
function of the RV (180 cc/m2 was the most commonly used volume threshold if RVEF was normal, 170 cc/m2 was the most
commonly used volume threshold if RVEF was mildly decreased, and 150 cc/m2 was the most commonly used volume threshold if
RVEF was moderately decreased). Those using volume thresholds to guide PVR referral were not statistically different from their
peers in terms of: level of ACHD experience, number of TOF patients seen, location of practice, exposure to CMR, access to cardiac
surgery or number of PVR operations performed per year at their primary practice setting.
Conclusion: There is variability amongst ACHD practitioners with respect to PVR referral for the asymptomatic patient post TOF
repair with significant PR and RV dilation. With increasing RV dysfunction, practitioners are more inclined to refer for PVR and are
less inclined to rely on CMR RV volume thresholds. The uncertainty regarding thresholds for PVR referral emphasizes the need for
a randomized controlled trial in this field.
31. Real-Time Three-Dimensional Transesophageal Echocardiography in the Intraoperative Assessment of Mitral Valve
Grewal J, Miller FA, Freeman WK, Abel MD, Click RL, Suri RM, Phil DK, Oh J, Pellikka PA, Mankad SV- Mayo Clinic, Rochester,
MN, United States
Background: Surgical mitral valve (MV) repair is the intervention of choice in the treatment of severe mitral regurgitation (MR).
Typically MV repair is accomplished after assessment of the MV anatomy using two-dimensional (2D) transesophageal
echocardiography (TEE) to delineate pathoanatomy and hence repairability. Three-dimensional (3D) TEE has been previously
shown to precisely identify MV pathology, however its mainstream use has been limited by prolonged acquisition times and the
need for off-line reconstruction. The aim of this study was to evaluate the feasibility of new generation real-time 3D TEE imaging in
the intraoperative assessment of MV pathology and to compare this novel technique to 2D TEE.
Methods: Thirty-one consecutive patients undergoing MV repair for MR were studied prospectively. Intraoperative 2D and real-
time 3D TEE examinations were performed employing a recently introduced TEE probe that provides real-time 3D imaging. The
etiology of MR by 3D TEE was assessed by expert echocardiographers blinded to the 2D TEE findings. Similarly, the 2D TEE
findings were assessed by experts blinded to the 3D TEE findings. Both were compared with the anatomic findings reported by the
Results: At the time of surgical inspection ischemic MR was identified in 16%, complex bileaflet myxomatous disease in 29% and
specific scallop disease in 55%. 3D TEE image acquisition was performed in a short period of time (60 + 18 sec), and was feasible
in all patients with optimal (39%) or good (39%) imaging quality in the majority of cases. There was a trend toward superiority in
identification of the predominant MV pathology with 3D TEE vs. 2D TEE (96% vs.89%, p=0.25). However, accuracy of 3D TEE
vs.2D TEE was superior in the diagnosis of A2, A3 and bileaflet disease (p< 0.05).
Conclusion: Real-time 3D TEE is a universally feasible method for identifying specific MV pathology in the setting of complex
disease and can be expeditiously employed in the intraoperative evaluation of patients undergoing MV repair. Specifically, it is
superior to 2D TEE in the identification of more complex anterior leaflet and bileaflet MV disease and hence could serve as an
important tool in guiding complex MV repair procedures in the future.
32. Increased Augmentation Index in Post-Coarctectomy Patients: Possible Explanation f or Increased Risk of Cardiovascular
Mackaij GJ, den Bogaard B, Van den Born BJH, Van Montfrans GA, Groenink M, Bouma BJ, Mulder BJM- Academical Medical
Centre Amsterdam, Netherlands
Background: Despite successful surgical repair, post-coarctectomy patients suffer from early onset cardiovascular disease (CVD),
which cannot fully be explained by complications such as restenosis and hypertension. Enhanced pressure wave reflections and
increased arterial stiffness have been proposed to explain the increased risk of CVD. We therefore assessed arterial wave reflections
and arterial stiffness in post-coarctectomy patients and matched controls non-invasively.
Methods: We examined 10 post-coarctectomy patients aged 29 ±7 yrs, 7 males, without significant restenosis and without
antihypertensive treatment. Ten healthy age and gender matched subjects served as controls (age 26 ±2 yrs, 6 males). Blood pressure
(BP) was measured with an oscillometric device after 15 min. rest. Radial artery waveforms were recorded non-invasively by
applanation tonometry using the Sphygmocor device.
Aortic augmentation index (AIx) was calculated using a validated transfer function and corrected for heart rate. Pulse wave velocity
(PWV) was measured between carotid and femoral arteries. All measurements were performed three times on the right and left
radial artery and were averaged.
Results: Post-coarctectomy patients had a higher right-sided systolic and diastolic BP than controls (134/75 ±8/10 vs. 124/65 ±7/5
mmHg, p <0.01), on the left side only diastolic BP was higher in patients (122/76 ±15/10 vs. 123/64 ±8/5 mmHg, p <0.01). AIx was
significantly increased on both the right (11.4±16.8 vs. -12.4±8.8, p <0.01) and left side (22.5±7.9 vs -10.5±10.4, p <0.01). PWV
showed no difference between patients and controls (right 5.7±0.9 vs. 5.8±0.9, p <0.70; left 5.5±0.7 vs. 5.7±0.8, p <0.44).
Conclusion: Augmentation index is increased in post-coarctectomy patients. The finding of normal arterial stiffness combined with
a distinct right to left difference in augmentation suggests that the enhanced wave reflection likely arises from early pulse wave
reflection on the reconstructed aorta despite the absence of significant restenosis. Non-invasive augmentation index measurement
might predict the risk of early onset cardiovascular disease.
33. BNP levels and assessment of exercise capacity with cardiopulmonary stress test in cyanotic adult patients with congenital
Trojnarska O, Oko-Sarnowska Z, Lanocha M, Gwizdala A, Grajek S- University of Medical Sciences, Poznan, Poland
Background: Heart failure remains the main cause of mortality in patients (P) with cyanotic congenital heart diseases (CHD).
Serum BNP level and cardiopulmonary exercise test (CPET) have a diagnostic and prognostic value in left heart failure, essential
pulmonary hypertension, cyanosis and some CHD. The goal of this study was the evaluation of exercise capacity using CPET in
adults with cyanotic CHD, measurement of serum BNP levels and analysis of the relationship between BNP level and CPET
parameters as well as determination of how these variables are affected by the degree of blood desaturation (SO2) and presence of
pulmonary hypertension (PH).
Methods: The study involved 53 (P) (20M) aged 19-65 years (39.4±14.3); 19 of them were operated on at the age of 1-32 (9.6±8.6)
years, which was 8-33 years earlier (20.8±7.6). Analyzed group 24 P: Eisenmenger syndrome, 16 P: single ventricle physiology, 4
P: transposition of the great arteries (Senning repair), 6: tetralogy of Fallot, 3: Ebstein anomaly. The controls: 32 healthy individuals
(16M) aged 26-59 years (40.7±9.9). Cyanosis: resting blood oxygen saturation (SO2) ≤ 90%. Secondary PH: Doppler
echocardiography -systolic right ventricular pressure >35 mmHg.CPET analyses included peak oxygen consumption (VO2max),
VE/VCO2 index, heart rate at peak workload (HRmax). Spirometry: forced vital capacity (FVC), forced expiratory volume in one
second (FEV1). BNP levels- immunoradiometric assay.
Results: 36 P- NYHA II, 13 P- NYHA III. Adults with CHDs and cyanosis had decreased exercise capacity compared to healthy
population: VO2max - 15.5±4.9 ml/kg/min vs. 31.6±7.1 ml/kg/min (p=0,00001); HRmax - 139.5±22.5 bpm vs. 176.6±12.1
(p=0.0001); peak systolic blood pressure 157.0±19.6 vs. 174.6±12.5 mmHg (p=0.00002); VE/VCO2 slope: 46.4±10.1 vs. 26.9±2.9
(p=0.00001), RQ: 1.0±0.04 vs. 1.1±0.07 (p=0.0001). FVC and FVC%: 3.1±1.1 l vs. 4.4±0.8 l (p=0.00001), FEV1 and FEV1%:
3.6±0.7 l vs., 2.5±0.9 l (p=0.00001). PH + group had worse exercise capacity than PH- group: VO2 max: 17.2±4.2 vs. 12.8±4.8
ml/kg/min (p=0.002), VE/VCO2: 43.7±11.1 vs. 50.9.9±6.4 (p=0.01), BPmax 161.2±21.3 vs. 150.8±15.0 (p=0.04), FVC: 3.46±1.05
l vs. 2.37±0.91 l (p=0.0002), FEV1: 2.9±0.8 l vs. 1.75±0.68 (p=0.00007). No differences between PH+ and PH- groups were
observed with respect to SO2: 80.4±7.3 vs. 82.5±4.3 (p=0.49). BNP level was higher in the study then in controls: 122.4±106.7 vs.
21.1±20.2 pg/ml, p=0.00001, and did not differ between PH+ and PH- P: 115.7±99.0 vs. 127.9±114.1 pg/ml (p=0.78). The
following correlations of BNP levels were obtained with: VO2 max (r=-0.389. p=0.006), FVC (r=-0.395, p=0.005), EFV1 (r=-0.386,
p=0.006), VE/VCO2 (r=0.369, p=0.009) and SO2 (r=-0.445, p=0.000003).
Conclusion: 1/Exercise capacity is significantly compromised in patients with CHDs and cyanosis.
2/ Serum BNP level is increased in this group of P being closely correlated with heart failure and severity of blood oxygen
desaturation while showing no significant relation with pulmonary hypertension.
34. The Utility of Stress-Echo Doppler to Unmask Post-Operative SVC Stenosis.
Patterson MWH, Sandor GGS, De Souza AM, Keiss M, Potts JE- BC Children's Hospital & St. Paul's Hospita1, Vancouver, BC,
Background: SVC stenosis (SVCs) is a known complication following atrial switch (AS) surgery for transposition of the great
arteries (TGA) or repair of sinus venosus atrial septal defect (ASD). The incidence of clinically significant SVCs is difficult to
ascertain as the symptoms may be non-specific and protean. We report the results of a stress echo-Doppler study to unmask SVCs.
Methods: The study group consisted of 38 patients (27 male), 30 were post-Mustard and 5 post-Senning for TGA, and there were 3
miscellaneous repairs. Patients underwent exercise testing a median of 20.5 years post-surgery. A total of 44 exercise tests were
performed, 25 in patients without intervention (NI), 9 pre-intervention (Pre-I) and 10 post-intervention (Post-I). Semi-supine cycle
ergometry was performed to volitional fatigue using a 20-40 watt, 3-minute step protocol.
All patients had Doppler velocities measured with the sample volume placed at the upper and lower ends of the SVC or baffle.
Intervention included 2 SVC angioplasties and/or 10 stents for symptoms or pacemaker-related concerns.
Results: Group # Tests Upper SVC Velocity (m/s) Lower SVC Velocity (m/s)
Pre-Ex Post-Ex Gradient Pre-Ex Post –ExGradient
NI 25 0.60 0.99 0.28 1.10 1.60 0.57
Pre-I 9 0.56 0.74 0.29 1.30 2.20 1 1.00 2
Post-I 10 0.63 0.93 0.27 0.94 1.66 0.77
Median values are reported; 1 P<0.05; 2 P<0.03
These differences were seen in the 6 patients who had both pre- and post-intervention studies, but the sample was small.
Conclusion: Stress Echo-Doppler demonstrated increased Doppler velocities and gradients distal to the stenosis in patients with
SVCs and was able to demonstrate the dramatic effect of intervention. Clinical findings need to be integrated with this data, as there
was an overlap of velocities and gradients in symptomatic and asympomatic patients.
35. Right ventricular contraction duration: a predictor for the response on bosentan treatment in patients with pulmonary
Duffels M, Hardziyenka M, Surie S, de Bruin-Bon HACM, Van Dijk APJ, Bouma BJ, Tan HL, Bresser P, Mulder BJM- AMC
Amsterdam, UMC Nijmegen, UMCU Utrecht, Netherlands
Background: Bosentan, a dual endothelin receptor antagonist, has been reported to have a positive effect on exercise capacity,
hemodynamics and survival in most, but not in all patients with various forms of pulmonary hypertension (PH). The six-minute
walk distance (6-MWD) is commonly used as a primary end-point. The purpose of this study was to identify specific
echocardiographic parameters to predict the effect of bosentan treatment.
Methods: Eighteen patients (9 patients with pulmonary arterial hypertension (PAH) due to congenital heart disease (CHD) and 9
patients with chronic thromboembolic pulmonary hypertension (CTEPH)) were included. Six-minute walk test and transthoracic
echocardiography at baseline and at one-year follow-up were performed. Patients with Down syndrome were excluded from this
Results: All 18 patients with pulmonary hypertension (age 57 years, range 35- 79 years, 33% male) used bosentan for at least one
year. Mean 6-MWD increased from 416±116 m to 448±128 m (p=0.02) after one-year treatment. Sixty-seven percent of the patients
(n=12) showed an increase in 6-MWD on bosentan treatment together with a decrease of echocardiographically measured
pulmonary vascular resistance (900±384 at baseline to 758±296 dyne*s*cm-5 (p=0.06). These patients had a significantly longer
right ventricular (RV) contraction duration at baseline (608±65 msec) compared to the 6 patients who did not improve on bosentan
treatment (515±21 msec; p< 0.01). A baseline RV contraction duration >550 msec, corrected for heart rate, was found to be a
predictor for a positive response on bosentan treatment (R=0.8, p<0.01). During one-year treatment, RV contraction duration tended
to decrease from 612±66 to 566±77 msec (p=0.1).
Conclusion: After one-year of bosentan treatment, 6-MWD increased in those patients with a RV contraction duration > 550 msec
at baseline. RV contraction duration can be used to predict the response on bosentan treatment in patients with pulmonary
36. Right atrial size relates to invasive markers of chronic diastolic dysfunction in an adult population with congenital heart
Do DH, Therrien J, Marelli A, Martucci J, Sebag IA- McGill University, Montreal, QC, Canada
Background: Little is known about the effects of chronic right ventricular (RV) dysfunction on the adaptive response of the right
atrium (RA). Because of the difficulty in determining RV diastolic function non-invasively, as an initial step, we tested the
hypothesis that RA size, measured by echocardiography, is a marker of chronic RV diastolic dysfunction, as determined by RV end
diastolic pressure (RVEDP) and mean RA pressure (RAP) measured by right heart catheterization.
Methods: We studied 28 consecutive adult patients with congenital heart disease (CHD), excluding patients with moderate/severe
tricuspid regurgitation, who underwent right heart catheterization and 2-D echo shortly thereafter, reviewing the 4-chamber view
from an apical acquisition. We measured: 1) maximal RA long-axis and short-axis lengths; 2) area; and 3) volume by the monoplane
area-length ellipsoid method (8x (area) 2/3π x L). We compared each of these measures to RVEDP and mean RAP.
Results: Of the 28 patients (mean age, 41±14 years), 15 had previous heart surgery, 10 had only a percutaneous intervention and 4
had previous surgery plus percutaneous intervention. Mean RVEDP = 9±5 mmHg, mean RAP = 9±4 mmHg and mean RA volume =
52±30 ml. RA long-axis, area and volume correlated well with RVEDP (R=0.72, p<0.001; R=0.72, p<0.001; and R=0.76, p<0.001,
respectively) and mean RAP (R=0.74, p<0.001; R=0.71, p<0.001; and R=0.72, p<0.001, respectively).
Conclusion: In adult patients with CHD, RA size represents a non-invasive marker of RVEDP. RA size relates to RVEDP even if
RVEP is normal. Further studies are needed to determine if increased RA size could be a precursor of RV dysfunction and serve as a
marker for the timing of surgery.
37. Outcome of Intra-Atrial Reentry Tachycardia Ablation Utilizing a Collaborative Approach Between Adult and Pediatric
Electrophysiologists: A Single Center Experience.
LaPage M, Rhee EK, Gleva MJ- Washington University and St Joseph's Hospital, Phoenix, AZ, United States
Background: Complex atrial anatomy in repaired congenital heart disease (CHD) can make ablation of intra-atrial reentry
tachycardia (IART) challenging. From 2001 to 2007 we utilized a collaborative approach for ablation of IART in adults with
Methods: We retrospectively reviewed the records of all adults with repaired CHD who underwent ablation for IART at our
institution between Jan 2001 and Jun 2007. Ablations were performed collaboratively with both adult and pediatric
electrophysiologists in attendance. Patients were excluded if no follow-up records were available or if ablation was immediately
unsuccessful (IART not ablatable). Patients were analyzed as 3 groups: Fontan, atrial switch (Mustard or Senning), and other
repaired CHD. The IART Clinical Severity Score (CSS), a cumulative index of symptoms and therapies, was calculated for patients
pre- and post-procedure at 3-6 months, and at most recent follow-up.
Results: There were 37 IART ablations on adults with CHD at our institution from 10-1-2001 to 7-1-2007. Nine patients were
excluded for unavailable follow-up records and 3 for unsuccessful ablation (1 from each group). Twenty-five patients were
included: Fontan (n=6), atrial switch (n=10), and other (n=9). Median f/u was 27 months (range 4-66; n=20). There were no
significant differences between groups regarding age (30.6 +/- 8.3 yrs, range 18.1 to 48.1), previous ablation attempts, mapping
technique, ablation time (55 +/- 35.6 min, range 8.4 to 145), or procedure time (311 +/- 84 min; range 173 to 525). Cavotricuspid
isthmus dependent IART was less common in Fontan patients compared to other groups (5.3% vs 36.8% vs. 26.3%; p = 0.16). Pre-
procedure CSS (4.2 +/- 1.1; range 2-7) was not significantly different between groups. Follow-up at 3-6 month (n = 19) showed
significant improvement (Δ -2.3 +/- 1.6; p = < 0.0001) in CSS (mean 1.7 +/- 1.9) with no difference between groups (p =
0.86). CSS remained improved at latest f/u (mean 1.0 +/- 0.28) compared to pre-procedure score (Δ -3.2 +/- 1.8, p = < 0.0001).
Conclusion: Successful outcomes for IART ablations, with acceptable procedure times, are achieved in adults with CHD and
complex atrial anatomy utilizing a collaborative adult / pediatric electrophysiologist approach.
38. Nationwide Survey of Care Facilities for Adults with Congenital Heart Disease in Japan.
Toyoda T, Shirai T, Tateno S, Kawasoe Y, Mizuno Y, Matsuo K, Niwa K- Chiba Cardiovascular Center, Chiba, Japan
Background: The numbers of adults with congenital heart disease (ACHD) are increasing rapidly. However, care programs for
those with ACHD have not been fully established. This study aimed to investigate the present condition of care for ACHD in Japan.
Methods: Questionnaires regarding status and resources of outpatient and/or in-hospital services and management of pregnancy in
ACHD patients were delivered 1033 training hospitals for board certified cardiologists. Precise data were collected from 448
Results: Although 407 hospitals (91%) followed at least one ACHD patient in outpatient clinic, only 14 hospitals (3%) had official
specialized outpatient clinic for ACHD. 344 hospitals (77%) had in-hospital patients with ACHD, however, only 6 hospitals (2%)
had >50 admitted patients per year. Surgery for ACHD was performed in 225 hospitals (50%) but 129 hospitals (57%) had <5 cases
per year. 153 hospitals (34%) had experience in the management of pregnant patients with ACHD but only 3 hospitals (2%) had >10
cases per year.
Conclusion: In Japan, most hospitals have been following up patients with ACHD. However, specialized care for patients with
ACHD has not been fully organized, especially for pregnant patients with ACHD. Early establishment of multi-disciplinary care
facility for ACHD is mandatory.
39. Pulmonary valve replacement following tetralogy of Fallot repair. When late is too late? Single centre experience.
Kadlec J, Weerasena NA, Watterson KG, Guerrero RR- Leeds General Infirmary, United Kingdom
Background: Improvement in intracardiac tetralogy of Fallot (TOF) repair is consistent with favorable long-term outcomes for
majority of patients. However, chronic pulmonary regurgitation may lead to right ventricle (RV) dilatation, dysfunction, arrhythmias
and sudden death. 15% of long-term survivals may require pulmonary valve replacement (PVR) to prevent such consequences.
Optimal timing of PVR is one of the most important predictor.
Methods: Retrospective data of patients, who underwent PVR following previous TOF repair between 01/01/03 to 31/12/07, were
collected from clinical notes and local database. Functional status, QRS duration, arrhythmias interventions, RV characteristics,
pulmonary valve regurgitation (PR) and magnetic resonance were considered prior to and after surgery and outcomes of surgery
Results: 29 patients underwent PVR in mentioned period of time. PVR was performed at mean age of 28.1+/-10.3 years (range 16–
61.7), 22.2+/-7.7 years (12-2.8) after TOF repair, which was performed at mean age of 5.7+/-6.8 years (1.1-38). As regards
preoperative data, functional status was NYHA class I, II, III in 2 (6.9%), 21 (72,4%) and 6 (20.7%) patients respectively. 19
(65.5%) patients experienced palpitations and 2 (6.9%) required permanent pacemaker. QRS mean was 166+/-33 ms and QRS>180
ms was found in 9 (31.0%) cases. Echocardiography showed severe PR in 28 (96.6%), moderate to severe RV dilatation in 28
(96.6%), moderate/severe RV impairment in 14 (48.3%) and aneurysmal RVOT in 11 (37.9%). RV estimation by magnetic
resonance was done in 11 (37.9%) cases and started to be more frequent during the last 2 years.
Postoperative assessment showed functional status improvement in majority of patients, NYHA class I in 24 (85.7%), II 4 (14.3%)
and III 0 (0%) cases (P<0.001). QRS changed to mean 162+/-29 (P>0.1). Internal cardioverter defibrillator was implanted in 10
(34.4%) patients after PVR (indicated due to preoperative ventricular tachycardia), 6 (60%) of them had QRS>180 ms. Noticeable
reduction of RV volume was observed on echocardiography in 11 (39.3%; P=0.003) and slight improvement of function in 9
(32.1%; P>0.5) patients, which led to overall normal/mildly dilated RV in 8 (27.6%) and normal/mildly impaired RV function in 14
Operative data showed that homograft was chosen in 6 (20.7%) and bioprosthesis in 23 (79.3%) cases, and 2 (7.1%) patients had
additional atrial cryoablation. One patient (3.4%) died on table and 3 (10.3%) had serious postoperative complications (twice
prosthetic bacterial endocarditis, once distal homograft anastomosis stenosis), out of which 2 required early reoperation.
Conclusion: This study shows that preoperative moderate/severe RV impairment in 14 (48.3%) patients did not significantly
improve after PVR, reduction of RV volume in these patients was only mild and defibrillator need high. Thus, patients with
established significant RV impairment prior to surgery are indicated too late. We assume, that recent standard introduction of
magnetic resonance will help improve timing, before RV dysfunction develops. Promising RVOT reduction plasty and cryoablation
surgery were also introduced and need later evaluation.
40. Evaluation of exercise capacity with cardiopulmonary exercise testing and type B natriuretic peptide in adult patients with
congenital heart disease.
Trojnarska, O, Oko-Sarnowska Z, Lanocha M, Gwizdala A, Grajek S - University of Medical Sciences, Poznan, Poland
Background: BNP level and cardiopulmonary exercise test (CPET) are validated predictors of diagnosis and prognosis in patients
(P) with heart failure- the main clinical problem in patients with congenital heart disease (CHD). The aim of this study was the
evaluation of exercise capacity - CPET and measurement of serum BNP levels in adults with CHD, in the entire study group and in
the subgroups of individual anatomical anomalies; moreover analysis of the relationship between BNP and exercise performance.
Methods: The study group: 265 P (136M) aged 19-65 years (34.4±11.6), 163 P were operated on at the age 1-42 years (9.2±7.3),
163 of them were, 9-34 years earlier (mean 21,8±7,4). 72P- operated Coarctation of Aorta (CoAo), 62P- Operated tetralogy of Fallot
(ToF), 28P- Ebstein anomaly, 26P- patent ASD, 24P- Eisenmenger syndrome, 20- unoperated or palliated complex cyanotic lesions,
14P- transposition of the main arteries after Sennig operation (TGA), 11- corrected transposition of the main arteries (CCTGA), 8P-
Fontana physiology. The controls: 39 healthy individuals (17M) aged 23-58 years (35.8±9.3). CPET: peak oxygen consumption
(VO2max), VE/VCO2 index, heart rate at peak workload (HRmax), forced vital capacity (FVC), forced expiratory volume in one
second (FEV1). BNP levels- immunoradiometric assay.
Results: 207P - NYHA I, 47P- NYHA II, 11P - NYHA III. Adults with CHD had decreased exercise capacity compared to controls:
VO2max: 23.2±6.9 vs. 33.6±7.2 ml/kg/min (p=0,00001); HRmax: 161.1±33.8 vs. 179.6±12.3 bpm (p=0.00001); VE/VCO2 slope:
35.7±9.7 vs. 26.3±3.1 (p= 0.00001); FVC: 3.8±1.1 vs. 4.6±0.7 (p=0.00003), FEV1 3.0±0.8 vs. 3.7±2.4 l (p=0.00001).
Comparison of VO2 in individual CHD groups (ml/kg/min) (mean±SD): CoAo: 26.8±6.6. ToF: 24.9±5.7, ASD: 24.6±4.8, CCTGA:
24.2±6.6, Ebstein anomaly: 22.3±5.0, TGA: 21.1±5.9, Fontan physiology: 19.5±3.8, unoperated or palliated complex cyanotic
defects: 15.9±3.4, Eisenmenger syndrome: 12.8±4.8 (ANOVA p=0.00001).BNP was higher in the study group then in controls:
55.4±67.5 vs. 13.9±13.7 pg/ml p=0.00001. Comparison of BNP levels in CHD subgroups (pg/ml) (median, min-max): CoAo
24.8(1.4-299.9), Ebstein: 26.7(5.2-178.2), ToF: 29.3(6.2-159.4), TGA: 32.8(15.2-321.4), ASD: 35.7(13.8-276.1), CCTGA:
42.1(11.3-180.2), Eisenmenger 75.6(14.2-343.9), Fontan: 95.5 (27.9- 278.8), cyanotic defects: 120.7(28.8-456.5) (ANOVA
p=0.00001).BNP levels were 35.7±34.0, 94.1±78.0, 225.6±114.89 pg/ml in patients at NYHA class I, II, III, respectively (NYHA I
vs. II: p=0.00001, I vs. III p=0.00001, II vs. III p=0.05).We observed the significant correlations of BNP levels with: VO2max (r=-
0.525, p= 0.0001), VE/VCO2 (r=0.447, p=0.00001).
Conclusion: 1/ Exercise capacity in adults with CHD is significantly compromised which is the most prominent in CHD patients
with pulmonary hypertension and cyanosis.2/ BNP level is increased in this group of patients being closely correlated with exercise
capacity and is highest in patients with cyanotic CHD.
41. Evaluation of exercise capacity with cardiopulmonary exercise testing and BNP levels in adult patients with morphologically
right ventricle in systemic position.
Trojnarska O, Oko-Sarnowska Z, Lanocha M, Gwizdala A, Grajek S - University of Medical Sciences, Poznan, Poland
Background: Heart failure remains the main cause of death in patients with morphologically right ventricle being a systemic
ventricle, as in patients with both transposition of the great arteries (TGA) surgically corrected via Senning or Mustard procedure or
congenitally corrected transposition of the great arteries (CCTGA). Concurrently, there is reliable and feasible echocardiographic
assessment of right ventricle function.
Serum B type natriuretic peptide (BNP) level and cardiopulmonary exercise test (CPET) are objective diagnostic and prognostic
tools. The aim of this study was the evaluation of exercise capacity and measurement of serum BNP levels as well as analysis of the
relationship between BNP levels and CPET parameters in asymptomatic or minimally symptomatic adults with morphologically
right ventricle being a systemic ventricle.
Methods: The study comprised 26 patients (P) - 15 P: with TGA after Senning operation, and 11 patients with CCTGA, (13M) aged
19-58 years (30.3±10.2). TGA patients were operated at the age of 1-14 years (4.3±3.7), and 18-30 years ago (21.6±4.2) CCTGA- 3
patients operated at the age 11-33 years (20,7±11,2). The control group: 22 -healthy individuals (14M) aged 26-59 years (40.7±9.9).
Maximum exercise cardiopulmonary test - modified Bruce protocol. Analyses: peak oxygen consumption (VO2max), VE/VCO2
index, and heart rate at peak workload (HRmax). Spirometry: forced vital capacity (FVC), forced expiratory volume in one second
(FEV1). BNP levels were determined using immunoradiometric assay.
Results: 19 P- NYHA I, 7 P- NYHA II. Patients with TGA and CCTGA had significantly reduced exercise capacity compared to
healthy population: VO2 max- 22.4±6.3 ml/kg/min (55.6±11.9%) vs. 34.2±7.4 (89.4±16.0%) (p=0.00001, p=0.00001, respectively);
HRmax: 155.4±31.8 bpm (83.8±14.6%) vs. 187.7±15.9 bpm (97.8±7.9%), (p=0.00002, p=0.0001, respectively); VE/VCO2 slope:
33.7±6.1 vs. 25.8±3.3 p= 0.00001; RQ: 1.0±0.04 vs. 1.1±0.07 (p=0.001); FVC and FVC%: 3.8±0.9 vs. 4.6±1.1 and 96.4±15.4% vs.
104.1±9.6% (p=0.03, p=0.04, respectively); FEV1 and FEV1%: 3.2±0.7 vs. 3.7±0.9 l, and 88.3±18.9% vs. 99.1±11.6 (p=0.02,
p=0.02, respectively).BNP was higher in the study group then in controls: 62.2±72.0 vs. 10.9±8.2 (pg/ml) p=0.00001. Among all
cardiopulmonary parameters BNP correlated significantly with: HRmax r=-0,505, p=0.008 and HR% r=-0.498 p=0.009.
Additionally significant positive correlation was found between VO2 and HR max: r= 0.558 p=0.003.
Conclusion: 1) Despite subjectively good self-evaluation, exercise capacity in adult patients with morphologically right ventricle in
systemic position is compromised. 2) Serum BNP levels are increased in this group of patients being closely correlated with
chronotropic response, reflecting the cardiovascular capacity in this population.
42. Gender and outcome in adult congenital heart disease.
Verheugt CL, Uiterwaal CSPM, Van der Velde ET, Meijboom FJ, Pieper PG, Vliegen HW, Van Dijk APJ, Bouma BJ, Grobbee DE,
Mulder BJM - Academic Medical Center, Amsterdam, Netherlands
Background: Gender differences in prevalence of congenital heart disease at birth have been widely documented. The objective of
this study was to determine whether gender influences the risk of complications in adult patients with congenital heart disease.
Methods: From the CONCOR registry database (van der Velde, Eur J Epidemiol. 2005:20:549-57) 7,414 patients were identified.
Logistic regression was used with complication (yes/no) as outcome and gender as predictor. All analyses were adjusted for age and
for the most frequently underlying congenital heart defects per outcome. Results are expressed as odds ratios (OR) and 95%
confidence intervals (CI). Confidence intervals not including 1 are considered statistically significant.
Results: The figure shows that, compared to men, women had a 33% higher risk of pulmonary hypertension (OR=1.33, 95%CI
1.07-1.65, p=0.01), a 33% lower risk of aortic complications (OR=0.67, 95%CI 0.50-0.90, p=0.007), a 47% lower risk of
endocarditis (OR=0.53, 95%CI 0.40-0.70, p<0.001), and a 55% lower risk of ICD (OR=0.45, 95%CI 0.26-0.80, p=0.006). Further,
Women had a borderline statistically significant 12% lower risk of arrhythmia (OR=0.88, 95%CI 0.77-1.02, p=0.08).
Conclusion: The risk of several major cardiac outcomes in adult patients with congenital heart disease is determined by gender.
43. Are cyanotic patients protected against atherosclerosis?
Duffels M, Mulder KM, de Groot E, Bouma BJ, Hoendermis ES, Van Dijk APJ, Mulder BJM - AMC Amsterdam, UMC Groningen,
UMC Nijmegen, Netherlands
Background: It has been hypothesized that the cyanotic state is accompanied by a reduction of atherosclerotic risk factors.
Therefore, cyanotic patients might be protected against atherosclerosis. The purpose of this study was to investigate the long-term
effects of cyanosis on the development of atherosclerosis by determining risk factors such as fasting serum lipid levels and by
measuring the carotid intima-media thickness.
Methods: Adult patients with a cyanotic heart defect and a sex and age matched control group were selected for this study. Risk
factors for atherosclerosis were evaluated on medical history, fasting serum lipid level, serum platelet count, serum bilirubin level,
serum thrombocyte level, blood pressure and transcutaneous saturation. As a measure for atherosclerosis, carotid intima-media
thickness was assessed non-invasively using ultrasound. Risk factors for atherosclerosis were compared between cyanotic patients
Results: Currently, 44 cyanotic patients (24 males and 20 females, mean age 37, range 18-60 years) and 39 age and sex matched
controls are included. Eighty-four percent of the patients (n=37) had the Eisenmenger syndrome. Mean transcutaneous saturation of
the cyanotic patients was 84±7%.
Cyanotic patients showed a reduction of risk factors for atherosclerosis compared to controls; cyanotic patients had less often
elevated cholesterol levels (58% vs. 73%), significantly lower thrombocyt levels (152±65 vs. 252± 63 10E9/L; p<0.01), higher
bilirubin levels (20±12 umol/L vs. 14±6 umol/L; p<0.01), and lower systolic blood pressure (119±18 vs. 128±13; p=0.01). Carotid
intima-media thickness, adjusted for age, gender and smoking was not significantly decreased (Beta= -0.7, p=0.5).
Conclusion: Our results suggest that cyanotic patients might be protected against atherosclerosis due to a reduction of various risk
factors. However, carotid intima media thickness was not significantly decreased.
44. Trabeculations and papillary muscles have a clinically important influence on measured systemic right ventricular function
by means of cardiovascular magnetic resonance imaging.
Winter M, Bernink FJ, Groenink M, Bouma BJ, Van Dijk APJ, Helbing WA, Tijssen JGP, Mulder BJM - Academic Medical Center,
Background: The influence of trabeculations and papillary muscles on cardiovascular magnetic resonance (CMR) measurements is
marginal in individuals with normal cardiac anatomy. However, the abundance of these structures in patients with a systemic RV
could have a clinically important impact on measured volumes and function. The purpose of our study was to evaluate the influence
of trabeculations and papillary muscles on measured systemic right ventricular (RV) volumes and function and on reproducibility by
means of CMR.
Methods: Twenty-nine patients (mean age 35 ± 12.4 years) with a systemic RV (12 with congenitally corrected transposition of the
great arteries (TGA) and 17 with atrially switched TGA) underwent CMR. We compared systemic RV volumes and function using
two different analysis protocols; once trabeculations and papillary muscles were included in the ventricular volume, once they were
Results: Including trabeculations and papillary muscles in the ventricular volume resulted in higher values for systemic RV end-
diastolic volume (mean difference 28.7 ± 10.6 ml, p<0.001) and for end-systolic volume (mean difference 31.0 ± 11.5 ml, p<0.001),
compared to subtracting structures. Values for ejection fraction were significantly lower (mean difference -7.4 ± 3.9%, p<0.001).
Including structures resulted in better interobserver reproducibility for ejection fraction (p<0.01).
Conclusion: Trabeculations and papillary muscles have a clinically important influence on measured systemic RV volumes and
function by means of CMR. Including structures in the ventricular cavity leads to better reproducibility and shorter analysis time,
and should therefore be the method of choice in daily practice.
45. Impaired renal function in Eisenmenger's syndrome.
Jensen A, Iversen K, Vejlstrup N, Sondergaard L - Kardiologisk klinik B, Rigshospitalet, Denmark
Background: It is described in the literature that patients with cyanotic heart disease (CCHD) are at risk of developing nephropathy.
Previous studies have demonstrated that the kidneys of patients with CCHD may show histological lesions. Clinical studies have
reported proteinuria, reduced renal plasma flow, and a decreased glomerular filtration rate (GFR). Why some of the patients with
CCHD develop nephropathy is uncertain, but it seems from the literature that elevated hematocrit and duration of cyanosis are risk
factors. Though nephropathy has been reported in patients with CCHD, few studies have addressed the size of the problem. We
therefore decided to examine our patients with Eisenmenger's syndrome to evaluate renal function.
Methods: Twenty patients with Eisenmenger's syndrome (13 women), with a median age of 44 (range 33 - 73) years with median
hematocrit and arterial hemoglobin oxygen saturation levels of 59 (range 32 - 74) and 79 (range 63 - 93) percent, respectively, were
characterized with plasma-creatinine, 24 hours urine protein excretion, urine reagent strips, GFR (Cr EDTA), 24-hours ambulatory
blood pressure measurement, and a CT scan of the kidneys.
Results: All patients had normal plasma creatinine levels (median 72 (range 44 - 90) mmol/L). Two patients were known with
arterial hypertension, but were medicated, and all patients had blood pressures below 145/95 mmHg. None had signs of urinary tract
infection. 24-hour urine collection revealed that 5 patients had proteinuria (>300 mg), and the remaining 15 patients had
microalbuminuria (30 – 300 mg). GFR was reduced (corrected for sex, age, and body surface area) in 9 patients corresponding to a
proportion of 45% (34 to 56%; 95% confidence interval). CT scans did not show any pathological signs, except in two patients
where the changes could be due to either earlier renal infection or thromboembolism.
We did not find any connection between impaired renal function and age or hematocrit.
Conclusion: Almost half of our Eisenmenger patients had moderately reduced glomerular filtration rate even though they had a
normal creatinine level. All of our patients had microalbuminuria, and one fourth had proteinuria, an early sign of glomerulare
injury. Neither urinary tract infection, nor renal infarction, nor systemic arterial hypertension could explain the renal impairment.
We intend to follow GFR in all patients with cyanotic heart disease prospectively.
46. Is Spectrum of Complexity of Congenital Heart Defects In Developing Countries Different From Developed? Special
Reference To Some Unusual Complex Case Scenarios: A Single Centre Experience.
Mahajan V, Rajesh S, Pradipta A, Subeeta B, Anil B, Dinesh M- Batra Hospital and Medical Research Centre, India
Background: Managing CHD in India or any developing country is more challenging as the resources and infrastructure are
abysmally limited. Pediatric Cardiac centers providing care at par with centers around the globe are present but number is limited
considering the enormous volume of patients. There is hence undue delay in detection of CHD resulting in delay in referrals and
Methods: In the present review we analyzed the records of newly diagnosed patients with CHD admitted at our center for
stabilization or diagnostic work up or interventional / surgical procedure. The center gets referral from both privileged and
underprivileged areas. The patients were grouped into 2 based on whether they came from remote areas lacking good medical
facilities or financially poor strata (underprivileged) and privileged ones. We gave special reference to unreported / unusual case
scenarios as they are seen in circumstances peculiar to developing countries but at the same time provide us with deeper insight into
pathophysiology of CHDs.
Results: The total number of patients analyzed were 765 in last 30 months (underprivileged group – 356, Privileged - 409). The age
range was from 1 day to 75 yrs. The mortality rate of operated patients was 2.2 %. The percentage of patients detected and referred
late was 321/ 765 (42 %). The incidence of atypical presentation was 28/321 (8.7 %) in patients presenting very late. There was
significant difference in spectrum of complexity of CHD between the patients coming from underprivileged area versus those from
privileged areas (P < 0.05). The patients presenting late were mostly from underprivileged areas of the country or neighboring
countries viz. Pakistan, Bangladesh, Afghanistan and Guyana. The clinical scenarios included the following:
a. Transposition of Great Vessels, Intact ventricular septum, Large ASD (post BAS), systemic PA pressures, Prepared Left
ventricle at 8 yrs – Partial Balloon Occlusion of atrial septal defect predicted operabilty by reducing PA pressures and
b. Large congenital pseudoaneurysm of aorta associated with PDA, Coarctation of Aorta, Dysfunction LV – Suspected on
routine Echocardiography and confirmed on CT angiography. Operated successfully on Circulatory arrest.
c. Balloon Dilatation by Fogarty Embolectomy catheter of left bronchial stenosis in a ventilator dependent post operative
patient under fluoroscopy (ALCAPA with severe LV Dysfunction at 10 months)
d. Hypersplenism secondary to congestive splenomegaly (massive spleen) in a severely cyanosed tetralogy patient (22 yr
old) reversing 5 days after Blalock-Taussig Shunt.
e. Late presenting Ventricular Septal defects with Early Eisenmenger Syndrome – Combination of PA Banding plus
sildenafil possibly changes natural history
f. Tubercular Aortoarteritis in Infancy – Balloon dilatation of descending aorta
g. ALCAPA presenting at 58 yrs detected on coronary angiogram with admitting diagnosis of CAD.
Conclusion: The present review reveals that developing countries face the problem of late presentation of CHD especially in
underprivileged and underserved areas. The disease spectrum understandably is different and case management has to be guided by
financial implications and viability. At the same time these challenges reveal unusual case scenarios with diverse and peculiar
physiology. These clinical scenarios require newer innovative strategies and newer interventional and surgical modalities to achieve
47. Nursing Consultations for Pregnant Women With Heart Disease.
Kikkenborg berg S, Rasmussen G, Ostergaard L, Pedersen PU- Copenhagen University Hospital, Denmark
Background: Women with heart disease are, in most cases, able to become pregnant and give birth. Often, they need close follow-
up by a team consisting of a senior cardiologist, consulting gynecologist, specially educated midwife and CHD-nurse.
In 2004 such a set-up was established in our outpatient clinic. The purpose of this study is to look into the content of the nursing part
of the program. What are the demographic characteristics of the patients, and what topics are addressed during the nursing
Methods: Patients saw the CHD-nurse after consultation with the physicians. At the conclusion of the nursing consultation, the
nurse filled out a self-developed form for each patient documenting their demographic characteristics and data relating to which
topics the nurse found to be addressed during the consultation.
Data was collected for almost three years and processed using the statistical program SPSS 10.0.
Results: There were 338 consultations.
• The most common diagnoses were tachycardia (30 %), cardiomyopathy (9 %) and mitral valve regurgitation (7 %).
• 30 % has previously had heart surgery, 20 % a non-invasive procedure.
• In 90 % of the consultations the women were pregnant. 5 % were pre-pregnancy.
• 69 % were between 25 – 34 years old and 23 % were older than 35 years.
Content of the consultations:
• 11 % were given information about their heart condition.
• 10 % about medication.
• 13 % about prevention of endocarditis.
• 13 % instructions about delivery precautions.
• Under 10 % was given instructions about healthy living.
• 30 % were informed of potential symptom development during pregnancy.
• In 76 % of the consultations, psychosocial support was needed in coping with heart disease during pregnancy.
Conclusion: The role of the nurse in a multidisciplinary set-up is directed at supporting the patient cope with heart disease, because
pregnancy can become the circumstance in life, which makes normal coping insufficient.
Confronted with the seriousness of their heart condition, these women need psychosocial support from the CHD nurse; not in
discussing pregnancy, but in discussing managing and coping with heart disease during pregnancy.
48. Chronic Performance of a New Stylet-less, Catheter-Delivered Pacing Lead in the Adult with Congenital Heart
Karpawich P, Zelin K - Children's Hospital of Michigan, Wayne State, MI, United States
Background: The adult patient (pt) with congenital heart disease (ACHD) poses a unique set of problems with pacing lead implant
due to diverse structural anatomies as well as surgical repairs. In addition, pacing from traditional atrial appendage and ventricular
apical implant sites may adversely affect chronic paced myocardial performance, which can be detrimental to pts with preexisting
structural abnormalities. Alternate site pacing from more potentially optimal sites, resulting in improved paced myocardial
performance, can be technically challenging or nearly impossible with standard stylet-guided leads. The recent introduction of a new
flexible catheter-delivered, styled-less, steroid-eluting, 4.1 Fr diameter 3830 lead and Select Secure ™ catheter-delivery system
(Medtronic, Inc) can facilitate pacing at any pre-determined implant site. However, there is limited chronic lead performance
information of this new lead at these alternate implant sites in ACHD patients.
Methods: Using the new lead and catheter delivery system, 27 consecutive ACHD pts (19 male, 8 female), ages 17-48y (median
24), weight 45-104kg (median 60) received 37 leads (23 atrial, 14 ventricular) and followed from 1-30 months (median 18) post
implant. Repaired CHD included D-transposition (DTGA) (n = 11), septal defects (n=4), single ventricle/Fontan (n=3), aortic
stenosis (n=1); also congenital AV block (n=5), cardiomyopathy (n= 3). DTGA pts included 5 concomitant Mustard atrial baffle
devices (1 Amplatzer™ closure for baffle leak and 4 stents for obstruction). Among these pts, leads were implanted through the
stents or following the Amplatzer™ implant.Based on hemodynamic measurements (ventricular contractility indices dP/dt,
pressures and paced QRS duration), the most optimal alternate pacing locations included high septal right atrium (n=13), high left
atrium (n=8), right ventricular septum (n=9), left ventricular septum (n=7).
Results: Regardless of CHD anatomies or surgeries all pre-selected implant sites were readily achieved with the delivery catheter
with minimal fluoroscopy time (0.6-8 minutes (Median 2). There were no implant complications. One lead was dislodged within a
few weeks post implant due to pt trauma and easily repositioned. Chronic pacing and sensing indices showed lead stability and no
dislodgements. Chronic thresholds remained stable as well as lead impedances (#: p<0.5):
Site N R/P Implant Implant
(mv) Threshold (v) Impedance Ω
RA septum 13 3.5± 0.5 0.5±0.05 770±36
LA high 8 3.2± 0.5 0.8±0.1 1036±70
RV septum 9 12.5± 1.4 0.5±0.2 930±53
LV septum 7 12.2± 1.5 0.8±0.3 950±50
Chronic Chronic Chronic
RA septum 13 3.4± 0.5 0.4±0.03 560±36
LA high 8 3.6± 0.6 0.5±0.1# 730±60#
RV septum 9 10.6± 1.0 0.4±0.05 580±20#
LV septum 7 9.0± 1.0 0.5±0.04# 690±40#
Conclusion: In spite of diverse congenital anatomies, surgeries and the use of intravascular devices, the new catheter-delivered lead
facilitates implant with minimal time and technical skills as well as exhibits chronic stable performance indices in the ACHD
49. Cardiac Reserve a Predictor for Future Systemic Right Ventricular Function?
Winter M, Kumar S, Bouma BJ, Tulevski II, Groenink M, Mulder BJM - Academic Medical Center, Amsterdam, Netherlands
Background: Patients with a systemic right ventricle (RV) are often confronted with ventricular dysfunction. Moreover, these
patients are known to have an impaired cardiac reserve. The purpose of our study was to evaluate the predicting value of an
impaired cardiac reserve for future cardiac function.
Methods: Nine patients (67% male; mean age 33, range 27 to 42 years) with a systemic RV (6 patients with an atrially switched
transposition of the great arteries (TGA) and 3 with a congenitally corrected transposition of the great arteries (ccTGA)) were
included in this study. Systemic RV ejection fraction (RVEF) was assessed twice with a 7-year time interval, by means of
Cardiovascular Magnetic Resonance (CMR) Imaging. At baseline cardiac reserve was determined using dobutamine stress CMR,
and was expressed as the increase in systemic RVEF after dobutamine infusion.
Results: Systemic RVEF decreased significantly during 7. 3-± 0.4 years follow-up (47.4% vs. 38,6%; P<0.01). Linear regression
analysis showed a significant correlation between cardiac reserve (after log transformation) at baseline and the decline in systemic
RVEF over time; r2=0.47, p < 0.05 (figure 1).
Conclusion: Patients with an impaired cardiac reserve show a more rapid decline in systemic RV function, compared to those with
an adequate reserve. Larger patient numbers are needed to confirm our data.
50. Blood Flow Measured by MRI at Rest and Exercise After Surgical Bypass of a Hypoplastic Aortic Arch.
Pedersen L, Pedersen TAL, Pedersen EM, Emmertsen K, Hjortdal VE- Skejby Hospital, Aarhus University Hospital, Denmark
Background: Hypoplastic aortic arch can be treated surgically with insertion of a bypass tube from the ascending to the descending
aorta. Blood flow after this repair has not yet been studied.
Methods: Seven patients (5 males) with median age 18 years (14 - 54) and median weight 79 kg (51 - 91) were studied 25 months
(6 – 68) following sternotomy with insertion of a hemashield tube (diameter 14 or 16mm) from the ascending aorta (midway
between the sinotubular junction and the take off of the first brachiocephalic vessel) to the descending aorta right above the
diaphragm. Five patients were on antihypertensive medication and all were normotensive on 24 hour blood pressure monitoring.
Seven sex and age matched normotensive healthy controls were also studied.
MR real time flow measurements were performed in the ascending aorta (above the sinotubular junction and below take off of the
tube graft), and simultaneously in the tube graft and in the descending thoracic aorta in a plane orthogonal to both the vertical
midway part of the graft and the descending aorta. Flow measurements were made over 5 to 6 seconds covering several respiratory
cycles. All complete heart cycles obtained during the 5-6 seconds were analysed. Measurements were performed at rest and at two
different exercise levels (0.5 and 1.0 Watt/kg) cycling on a MRI compatible ergometer. Flow to the upper part of the body was
calculated by subtracting descending aortic flow and graft flow from the ascending aortic flow. The study was approved by the local
Ethical Committee and informed consent obtained from all participants.
Results: Flow in l/min/m2 in patients and controls at rest and during supine lower leg exercise:
Rest 0.5 w/kg 1.0 w/kg
Asc. ao 3.1+/-0.7 4.6+/-1.0 5.5+/-1.1
Desc. ao 1.0+/-0.6 1.5+/-0.9 2.0+/-1.2
Graft 1.5+/-0.4 2.3+/-0.8 2.8+/-0.9
Upper body 0.7+/-0.3 0.8+/-0.4 0.8+/-0.3
Asc. ao 3.4+/-0.9 5.2+/-0.9 6.1+/-0.8
Desc. ao 2.5+/-0.9 4.3+/-0.7 5.1+/-0.6
Upper body 0.9+/-0.5 0.9+/-0.3 1.0+/-0.3
Flow in the ascending and descending aorta and in the tube graft in patients increased significantly with leg exercise. Flow was
higher in the graft than in the descending aorta in patients both at rest and during exercise. Descending aortic plus graft flow in
patients was similar to descending aortic flow in controls. The flow to the upper body was similar in the two groups and unchanged
during leg exercise.
Conclusion: An ascending to descending aorta tube graft bypassing a hypoplastic aortic arch provide adequate flow to the lower
body without upper body steal at rest and during supine leg exercise.
51. Heart Failure in Adult Mustard Patients: An Elusive Diagnosis – Or Is It?
Ebenroth E, Hurwitz R- Riley Hospital for Children, Riley, IN, United States
Background: The improved survival of pediatric patients with significant congenital heart disease necessitates proper identification
and treatment of heart failure (HF) in this population. Adult survivors of the Mustard operation for D-transposition of the great
arteries (DTGA) often have myocardial dysfunction, but identifying the patients in HF can be a challenge.
We studied a cohort of 35 patients operated 18-33 years earlier to determine modalities useful in the diagnosis of HF.
Methods: Clinical records and health information surveys were used to place each patient into one of the 4 NYHA classes. These
values were independently compared to exercise stress test data and radionuclide and echocardiographic assessment of right
ventricular ejection fraction (RVEF). B-type natriuretic peptide (BNP) levels were available for 5 patients.
Results: With the exception of VO2 max which was normal in 88% of patients in NYHA I, no other test was predictive of NYHA
class. Echocardiographic estimates of RVEF were the least helpful, with only 25% of patients in NYHA class I having normal
results. Two patients in NYHA class I (12%) had normal results for all three evaluated tests, while only one patient from each of
class II and III were had three normal results. There was no correlation between NYHA class and RVEF (R2 = 0.004) or VO2 max
(R2 = 0.018). In one patient 2 years after testing, severe HF symptoms developed. BNP at that time was 1348. Repeat BNP after
effective medical management was reduced to 481. Four other patients in NYHA classes I or II had BNP values of 39, 125, 313,
Conclusion: Heart failure in Mustard patients can be difficult to diagnose and requires multiple, serial studies. Patients without
symptoms are often found to have some degree of cardiac dysfunction or exercise limitation. Mustard patients that are apparently
doing well clinically can have incipient HF or decompensate rapidly. We suggest that all patients who have undergone the Mustard
operation should be considered to be at risk for HF and treated medically with afterload reduction and/or beta blockade while being
closely monitored for signs and symptoms of worsening HF.
52. Obstetric Outcomes in Pregnant Women with Congenital Heart Disease.
Ouyang DW, Khairy P, Fernandes SM, Landzberg MJ, Economy KE- Department of Obstetrics and Gynecology, Brigham and
Women’s Hospital, Harvard Medical School and 2Boston Adult Congenital Heart Service, Brigham and Women’s Hospital and
Children’s Hospital Boston, Boston, MA, United States
Objective: To determine what cardiac risk factors predict sustaining adverse obstetric events and to assess the impact of avoiding
Valsalva during the second stage of labor in pregnant women with congenital heart disease (CHD).
Methods: We conducted a retrospective cohort study evaluating outcomes in women with CHD who delivered between 1998-2005.
We examined baseline cardiac characteristics in a multivariate logistic regression model to assess which were associated with an
increase risk of adverse obstetric events. We also compared outcomes of women who avoided Valsalva versus those who were
allowed to Valsalva.
Results: The study included 65 women with 112 pregnancies. An adverse obstetric event occurred in 32.6% (n=32) of ongoing
pregnancies, the most common being preterm delivery (n=19), postpartum hemorrhage (n=13), and preterm premature rupture of
membranes (PPROM) (n=9). Of the preterm deliveries, only 10.5% (n=2) were due to spontaneous labor as compared to 42.8%
(n=8) secondary to PPROM and 47.4% (n=9) being indicated preterm deliveries. In multivariate analysis, there were no
independent cardiac predictors for sustaining an adverse obstetric event. Women who avoided Valsalva and had an assisted second
stage had increased rates of postpartum hemorrhage and 3rd and 4th degree lacerations and a trend towards a prolonged second stage;
however, they did not demonstrate an increased rate of cesarean delivery.
Conclusion: Although one-third of pregnancies were associated with an adverse obstetric outcome, these events could not be
predicted by baseline hemodynamic characteristics. Women with CHD did appear to be at increased risk of having PPROM and
preterm delivery. The routine practice of avoiding Valsalva and assisting delivery may increase obstetric complications.
53. Aortic root surgery and Marfan syndrome: a double headache!
Vis J, Gooiker DJ, Koppen H, Ferrari MD, Tijssen JGP Mulder BJM- Academic Medical Centre, Amsterdam, Netherlands
Background: Recently, an association between migraine, especially migraine with aura, and congenital abnormalities of the aorta
was suggested. Aim of this study was to investigate the prevalence of migraine in patients with Marfan syndrome (MFS) and to
identify risk factors.
Methods: Adult patients with MFS and an age and sex matched control group from the general population were administered a
clinical interview by telephone. Migraine was diagnosed according to the International Headache Society criteria.
Results: In this analysis, 97 MFS patients (mean age 39±13 years; 51% females) and 80 controls (mean age 42±13 years; 60%
females) were included. Significantly more MFS patients suffered from migraine (respectively 44% vs. 28%; p=0.02). In MFS
patients, migraine with aura was diagnosed in 37% in MFS patients and 10% in controls (p=<0.001). We found MFS to be an
independent risk factor for having overall migraine (OR 2.4; 95%CI; 1.3-4.7, p= 0.008) and for MA (OR 6.2; 95%CI; 2.6-14.7,
p<0.001) adjusted for gender. Thirty-five percent of the MFS patients underwent aortic root surgery. In MFS patients, aortic root
surgery appeared to be an independent risk factor for having overall migraine (OR 3.9; 95%CI; 1.4-10.6, p= 0.008) and MA (OR
4.5; 95%CI; 1.7-12.4, p= 0.03), both in men and in women.
Conclusion: Migraine prevalence, especially migraine with aura, is increased in patients with Marfan syndrome. A history of aortic
root surgery is a significant additional risk factor for having migraine and migraine with aura. Our findings correspond with the
suggested association of migraine and aortic complications.
54. Prevalence of Congenital Heart Disease in General Population of Taiwan from 2000 to 2006.
Wu HM, Wang JK, Huang SC, Huang SK- National Taiwan University Hospital, Taipei, Taiwan Bureau of National Health
Background: With advances in fetal diagnosis, the incidence, prevalence and the distribution of the types of congenital heart
disease (CHD) may vary from previous reports. This study was to investigate such profile in the general population of Taiwan, an
Asian country with a population of 23 millions.
Method: Health care records were collected from National Health Insurance (NHI) databases from 2000 to 2006. The NHI
databases contained health care data from >95% of all the hospitals in Taiwan and >95% of the population receiving health care
reimbursed by Taiwan National Health Insurance program. From this database, we selected patients who met criteria listed for CHD,
on the basis of the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code.
Results: The average of the annual CHD incidence was 34 per 1,000 live births, (ranged from 30-37). The distribution was
secundum atrial septal defect 34%, ventricular septal defect 26%, patent ductus arteriosus 11%, tetralogy of Fallot 3.6%,
transposition of great arteries 1.3%, anomalies of pulmonary valve 6.5%, congenital stenosis of aortic valve 1.1%, endocardial
cushion defect 1.7%, coarctation of aorta 1.3%, tricuspid atresia or Ebstein’s anomaly 0.6%, common ventricle or truncus 1.6%,
hypoplastic left heart syndrome 0.13% and others 10%. Patients with CHD older than the age of 18 years accounted for 23.3% of
whole CHD population and the distribution of the CHD was similar to that in the whole CHD population. But, the proportion of
patients increased to 5% for tetralogy of Fallot, decreased to 3% for anomalies of pulmonary valve, increased to 2% for congenital
aortic stenosis and decreased to 1.1% for common ventricle or truncus and hypoplastic left heart syndrome. Prevalence of CHD was
5.6 per 1000 persons and 1.3 per 1000 adults.
Conclusions: In Taiwan, the annual incidence of CHD was relatively higher than that from previous studies, but the incidence of
severe types of CHD declined with year. Most of the CHD patients might survive to adulthood, except those with single ventricular