Adult Congenital Heart Disease Program

Adult Congenital Heart Disease Program Division of Cardiology Dr. Simon Jackson Queen Elizabeth II Health Sciences Center An overview of Adult Congenital Heart Disease And….a practical approach to murmurs Objectives • To gain an awareness of the resources available for adults with congenital heart disease • To understand specific problems unique to the ACHD population – Medical complications of cyanotic heart disease – Psycho-social implications (impact of living with chronic disease) • To understand the principle of SBE prophylaxis, and which ACHD patients DO NOT require • To gain a practical approach to the assessment of murmurs Why talk about ACHD? • To increase your awareness of the complexity of care for these young patients • To review some of the complex psycho-social problems related to chronic illness and impact on a “normal” life • To learn the specific complications of long term cyanotic heart disease Adult Congenital Population • North America -800,000 adults with a congenital cardiac anomaly • Approximately 1400 new patients with significant congenital heart defects will become adults in Canada each year Adult Congenital Population: Subtypes 1. Those who have received curative surgery 2. Those who have had palliative procedures to prolong life and improve its quality 3. Those for whom no treatment is available and care is purely supportive 4. Those in whom a diagnosis and plan of action have not yet been established Goals Adult Congenital Heart Program • Provide comprehensive multi-disciplinary care to the Adult Congenital patient population of the Maritime provinces • Provide training opportunities in the area of Adult Congenital Heart Disease • Provide education and support for adult congenital heart disease patients, their families and their primary care physicians • Contribute to clinical research in ACHD Adult Congenital Heart Program QE II HSC • Three adult Cardiologists • One rotating pediatric Cardiologist • One Nurse - Coordinator Consultative Support • • • • • • Social work Psychology Genetic Assessment Obstetrics/Gynecology Electrophysiology CV Surgery Anesthesia QE II HSC ACHD 1400 1200 1000 800 600 400 200 0 1995 1996 1997 1998 1999 2000 2001 2002 # Patients QEII HSC ACHD Program 90 80 70 60 50 40 30 20 10 0 1995 1996 1997 1998 1999 2000 2001 2002 Caths Surgeries Pregnancies Deaths Non-Cardiac Issues of ACHD Population 1. 2. 3. 4. 5. 6. Young population with associated co-morbidities Chronic illness Pregnancy and contraception Vocational counseling Un-insurability Uncertainty of the future Patient Education • Despite its lifelong illness most adolescents and young adults with CHD have inadequate knowledge about their cardiac conditions. • Patients need appropriate information and education to enable independent decision-making about choices in care. Co-Morbidities • Syndromes • Post-pump complications – Stroke – Cognitive decline • Respiratory disorders • Neurologic sequlae Psychological Issues Adults with CHD face special challenges: • Employment • Inter-personal relationships • Assuming responsibility for their own health care • Family planning Psychological Issues • Long-term disturbances range from frank psychiatric disorders to subclinical distress and abnormalities in neurocognitive functioning secondary to: – chronic illness & disability – parental overprotection – ischemia/hypoxia from operations Uncertainty and Insurability • Many patients are un-insurable • Limits opportunities with work, business, owning a home • Reinforces concerns about mortality Pregnancy • Health of the mother • Health of the fetus • Risk of congenital disease in the offspring Pregnancy • Pregnant women with CHD should be managed by the patient's obstetrician and ACHD cardiologist concurrently. • Post partum, the patients with CHD may need ICU/monitoring facilities even for relatively minor procedures or uncomplicated deliveries QE II Health Sciences Pregnancies : 1995 - 2002 • 1995 - 17(MRC Pregnancy Study) • 1996 - 14 • 1997 - 4 • 1998 - 17 • • • • 1999 - 17 2000 - 21 2001 - 22 2002 - 17 1988-2001 Summary QE II HSC • • • • • • 81 pregnancies 57 Live births (70.4%) 13 lost to followup, 4 TA, 6 SA, 1 stillbirth 7 (12% of live births) congenital defects >20 (35% of live births) obstetrical events >22 (39% of live births) cardiac events Pregnancy: Contraindications 1. Pulmonary hypertension 2. Severe LV (systemic) dysfunction 3. Marfan syndrome with dilated aortic root (> 4 cm) 4. Cyanotic heart disease Contraception • OCP may be contra-indicated • IUD’s increased risk of bacterial endocarditis • Permanent sterilization: risk of the anesthetic Medical Issues 1. Endocarditis prevention 2. “Other” surgeries 3. Specific conditions 1. Eisenmenger’s syndrome Endocarditis • Endovascular infection “on” some structure within the heart, usually a heart valve • High morbidity and significant mortality Dental Care • Regular dental care often in a hospital setting is mandatory for adult patients with CHD to decrease the likelihood of infective endocarditis • Endocarditis prophylaxis is recommended for all dental work except for 'fillings above the gum line' Endocarditis Prophylaxis • Education and good dental hygeine • Cardiac conditions are stratified into – high – moderate – negligible risk categories 1997 ACC/AHA Recommendations High Risk • those who have prosthetic heart valves • a previous history of endocarditis • complex cyanotic congenital heart disease, or surgically constructed systemic pulmonary shunts or conduits 1997 ACC/AHA Recommendations • For oral or dental procedures the initial amoxicillin dose is reduced to 2 gm 1 hour pre procedure • Clindamycin 600mg is offered to penecillin allergic patients 1997 ACC/AHA Recommendations • For gastrointestinal or genitourinary procedures, the prophylactic regimens have been simplified: – High Risk: IV ampicillin 2gm plus gentamycin 1.5 mg/kg then po amoxil or iv ampicillin – Moderate risk: 2 gm po amoxil 1 hour pre Non-Cardiac Surgery • Performance of any surgical procedures in some adult patients with CHD carries a greater risk than in the normal population. • Evaluation in a referral centre prior to surgery is recommended • Specific concerns regarding SBE prophylaxis, air filters, risk DVT/PE and need for understaning of physiology of lesion Physiologic Consequences of Cyanosis • Secondary erythrocytosis is normal (Hb 200-220) • Stimulus is low oxygen saturation leading to increase erythropoetin leading to increased red cell mass • Compensation to increase oxygen carried in the blood Non-Cardiac Sequelae of Erythrocytosis and Cyanosis 1. Abnormal hemostasis, 2. Easy bruising, epistaxis, gingival bleeding 3. bleeding or traumatic bleeding (including peri-operative bleeding) 4. Spontaneous intravascular thrombosis Non-Cardiac Sequelae of Erythrocytosis and Cyanosis 1. Hyperuricemia with gout, urate nephropathy 2. Glomerular sclerosis, secondary to cyanosis, is usually manifest initially as proteinuria 3. Hypertrophic osteoarthropathy 4. Gallstones and cholecystitis Hyperviscosity • Viscosity of blood increases with disorders of increased cellular mass • “thick” blood “clogs up” capillaries leading to symptoms: – Headache, lethargy, confusion, vision change progressing to stroke • Removal of RBC’s is an effective treatment Care of the Cyanotic Patient • Iron deficiency (anemia) is a crisis! – Reduced oxygen carrying capacity and reduced deformability of the red cells leads to increased symptoms and possibly an increased risk of stroke. Plebotomy • Rarely required for the secondary increase in RBC mass in cyanotic heart disease • Should only be done with adequte replacement of volume • Can lead to iron deficiency anemia ACHD • Know SBE prophylaxis • The non-medical issues of ACHD • Physiologic effects of cyanosis Question 1 Aortic insufficiency produces a: 1. Systolic ejection murmur 2. Diastolic ejection murmur 3. Diastolic rumble 4. Diastolic decresendo murmur Question 2 Tricuspid insufficiency produces a: 1. Systolic ejection murmur 2. Systolic rumble murmur 3. Diastolic rumble 4. Systolic regurgitant murmur Question 3 Pulmonary stenosis produces a: 1. Systolic ejection murmur 2. Diastolic decrescendo murmur 3. Diastolic rumble 4. Systolic regurgitant murmur Question 4 Mitral stenosis produces a 1. Diastolic rumble 2. Systolic rumble 3. Systolic regurgitant murmur 4. Diastolic decrescendo murmur Practical Approach to Murmurs 1. Know what valves are open or closed in systole and diastole 2. Decide if the murmur is systolic or diastolic 3. Decide if the murmur character is: Ejection Regurgitant Decrescendo Rumble 4. Decide where is the murmur heard best 5. Put it all together Know what valves are open or closed in systole? • In systole (ventricles ejecting blood) – AV and PV are open and – the MV and TV are closed • In diastole (ventricles being filled) – MV and TV are open while – the AV and PV are closed Facts you need to know • Stenotic (narrowed) valves make noise when they should be normally open (not opened enough) • Regurgitant (leaky, insufficient) valves make noise when they should be closed (valves are leaking blood the wrong way) Is the murmur systolic? • Time the murmur with the pulse • Murmurs heard when the pulse can be felt are systolic Facts: Ejection Murmurs • Ejection murmurs are always systolic (blood is ejected in systole) • Ejection murmurs peak and (almost) always fall in intensity • This means they begin after S1 and end (almost) always before S2 • Ejection murmurs arise from the aortic valve or pulmonary valve (or less commonly from the LV or RV outflow tracts) Regurgitant Murmurs: • Regurgitant murmurs means blood is regurgitating (going the wrong way) • Regurgitant murmurs are high pitched (the flow is from an area of high pressure to an area of much lower pressure) • Systolic regurgitant murmurs are (almost) always holosystolic (= pansystolic) and begin with S1 and end with S2 • Examples are: – mitral insuffiency – tricuspid insufficiency. – A VSD is another cause. Facts: Diastolic Murmurs • Diastolic murmurs can be – “Decrescendo”: high pitch, intensity decreasing during diastole, due to insufficiency of AV or PV – “Rumbles”: low pitched, localized, heard with bell, related to low pressure flow across a narrowed valve, (mitral stenosis, tricuspid stenosis) Final Facts • Know the areas where the murmurs are heard best – Aortic stenosis – Pulmonary stenosis – Tricuspid stenosis – Mitral stenosis Aortic area Pulmonary area Tricuspid area Mitral area (apex) Final Facts • Know the areas where the murmurs are heard – Aortic insufficiency** Left sternal edge – Pulmonary insufficiency Pulmonary area – Tricuspid insufficiency Tricuspid area – Mitral insufficiency** Mitral area, axilla, rarely to aorta ** Not where expected Case 1: Putting it together • You hear a systolic ejection murmur loudest in the upper right sternal border • Ejection murmurs come when a valve is not opened properly (stenotic) • This is the aortic area • This is the murmur of aortic stenosis Putting it together: Case 2 • You hear a systolic murmur loudest in the apex which is regurgitant • What is regurgitant? What does it mean? • What valves should be closed in systole? • What area is this? • This is the murmur of mitral insufficiency Putting it together: Case 3 • You hear a diastolic murmur loudest at the apex which is low pitched, and localized. • What is another name for a low pitched murmur? What does it imply? • What valves should be open in diastole? • What area is this? • This is the murmur of mitral stenosis Putting it together: Case 4 • You hear a diastolic murmur loudest at the left sternal border which is high pitched and decreases in intensity during diastole. • What is another name this type of murmur? What does it imply? • What valves should be closed in diastole? • What area is this? • This is the murmur of aortic insufficiency Describe the murmurs for the following lesions • • • • Pulmonary stenosis Pulmonary insufficiency Tricuspid stenosis Tricuspid insufficiency How else do we sort out murmurs? • Associated findings (intensity of heart sounds, associated sounds, palpation of heart) • Radiation of murmur • Effects of respiration, dynamic maneuvers • Pattern recognition • Knowledge of the disease process effecting the heart