peripartum cardiotherapy

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					Peripartum Cardiomyopathy


      Matthew Voth M.D.
            WCGME
    Dept. of Ob/Gyn – PGY-1
        Case Presentation
N.A. 22 y.o. G1 P0 @ 40 WGA presented
to LDR with chief complaint: contractions

2/85/-1 on initial exam
3/90/-1 recheck 1 hour later
Admitted to BCC for Expectant
Management of Labor
            Antepartum
109 lbs on initial exam. Gained 27 lbs
during pregnancy
28 week Hgb 10.1. Pt unable to tolerate
Niferex during pregnancy
C/O back pain requiring prn Lortab
Otherwise unremarkable antepartum care
    Case Presentation cont..
Initial Vital signs: BP 134/78, P-60 R-16
Progressed along labor curve for several
hours with occasional variable decel.
Good BTBV, overall reassuring
At 0500 called to evaluate prolonged
deceleration, pt was rushed to OR for
emergent C/S.
           Emergent C/S
No complications   APGARS 8/9
EBL 1000cc         Tight nuchal cord
                   Pt. To recovery in
                   stable condition
         Postpartum Care
Hgb on admission 11.5 gm/dl
6 hours post-op 7.4 gm/dl
800cc LR bolus given
Typed and Crossed for 2 Units
Hbg rechecked 8 hours later, 6.8 gm/dl
500cc bolus given
       Postpartum Day #2
A.M. Hgb 7.4 gm/dl
Pt. Not tachycardic, BP’s stable 130’s/70’s
Urine output >100cc/hour
IV DC’d PPD #2
    Postpartum Care cont…
Pt. Remained asymptomatic.
Vital signs remained stable until PPD#3
4 consecutive BP’s >140/90 and HR >110
Pt. Tol PO well. IV not restarted
C/O Headache
PIH labs ordered - WNL
   Postpartum Care cont….
PPD #4, Hgb 7.4
BP 138/85, pt. Asymptomatic
Discharged home
         ER Visit PPD#7
4 days after dismissal pt. Returned to ER
with complaints of:
  Shortness of breath-more pronounced when
  lying down
  Chest heaviness when lying down
  Lightheadedness x 2 days
           Physical Exam
BP 143/100             PIH labs ordered
Pulse 83, regular      20 mg Lasix given in
RR 19                  ER
O2 sat 100% on 1L      Admitted to 3-WH
2+ edema LE’sL         Cardiology consulted
Lungs crackles heard   Dx: R/O
at bases bilaterally   cardiomyopathy
       Cardiology consult
EKG- normal
BMP – WNL
CBC – Hgb 8.1 gm/dl
TSH - WNL
Troponin I –WNL
BNP – 949 normal range (<100 pg/ml)
Echo – Dilated cardiomyopathy
  Cardiology Consult cont….
PE: reported an S3 gallop

Lasix 40 mg IV x1 then 20mg PO daily
Lisinopril 5mg PO x1 then 10mg PO BID
KCl 40mg PO x1 then 10 mg PO BID
Ativan 0.5mg PO prn
Daily I’s and O’s
            3-Women’s
Post admit day 1- pt reportedly much
improved. Breathing easier. Ambulating.
Voiding >90cc/hour.
Edema diminishing
Post admit day 2 – pt. Discharged home,
asymptomatic. Vital signs stable. 3 kg
weight loss.
Review of Cardiac Changes in
         Pregnancy
Increase in blood volume
  As early as 4th week
  10-15% at 6-12 weeks
  Rises rapidly thru 32-34 weeks then a modest rise

  Net result = 1100 – 1600 cc increase or 30-50% above
  baseline

*Lund et al. Am J Obstet Gynecol 1967; 98:393
                  Review cont….
Increase in TBV due to:
   Increased vascular capacitance
   Systemic vasodilation

….as opposed to pure blood volume expansion
Renin is increased and ANP decreased
(would suspect alternate with pure BV expansion)
Shier et al N Eng J Med 1988; 319:1127
              Review cont….
Elevation of CO rises 30-50 %
Due to 3 important factors:
  Preload is increased due to increase in TBV
  Afterload is reduced due to decreased SVR
  Maternal HR rises 15-20 bpm



Robson, et al. Am J Physiol 1989; 256:H1060.
*Chapman et al. Kidney Int 1998; 54:2056
      What is a Cardiomyopathy??
    Characterized by dilation and impaired
    contraction of one or both ventricles.
    Affects systolic funtion
    Pt. May or my not develop overt heart
    failure.

*Richardson et al. Circulation 1996 93:841
                                     Cont…..
    Overall responsible for               Common Sx:
    10,000 deaths and                       Progressive dyspnea
    46,000                                  with exertion
    hospitalizations each                   Impaired exercise
    year                                    capacity
                                            Orthopnea
    Wide age range 20-60
                                            Paroxysmal nocturnal
                                            dyspnea
                                            Peripheral edema
*Dec et al. N Engl J Med 1994; 331:1564
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*Felker et al. N Engl J Med 2000; 342:1077
 Peripartum Cardiomyopathy
4% of all
cardiomyopathies
1:3000-4000 preg.
Dilated
Cardiomyopathy
  Should we be concerned??
Yes!
CDC Pregnancy Related Mortality
Surveillance 1991-1999
  Leading Causes of Maternal Mortality:
    Embolism – 20%
    Hemorrhage – 17%
    Hypertension – 16%
    Peripartum Cardiomyopathy- 9%***
               Etiology
Multiple studies have attempted to elucidate
a distinct etiology…..all have failed
Theories:
  Myocarditis
  Abnormal Immune Response
  Genetics
  High postpartum salt intake
                Myocarditis??
Nairobi Study1986
  11 African women with PPCM
  Endocardial biopsies done on all eleven
     5 showed evidence of “healing myocarditis”
       – Presence of inflammatory cells
       – Necrosis
       – Fibrous remodeling
  9 patients finished study
     75% of myocarditis group developed persistent heart failure
     80% of patients without myocarditis improved

   *Sanderson et al. Br Heart J 1986: 56:285
           Myocarditis? Cont…
Another study:
    84 women with cardiomyopathies
    14 diagnosed as being PPCM
        29% of patients with PPCM were found to have
        myocarditis
        Only 9% of idiopathic CM related to myocarditis


*O’Connell et al. J AM Coll Cardiol 1986; 8:52
         Myocarditis? Cont….
3rd Study:
18 patients with PPCM
  14 due to myocarditis
  10 of these received immunosuppressive Tx over 6-8
  weeks, then tapered over 6-8 weeks
      9 of 10 improved on therapy
      However, 4 of 4 not receiving therapy also improved


   *Midei et al. Circulation 1990; 81:922
        Myocarditis? Cont….
1994 Retrospective study
  34 patients diagnosed with PPCM
  Researches found lower incidence of
  myocarditis than previously reported
      8.8 % due to myocarditis


  Why the discrepancy??

* Rizeq et al. Am J Cardiol 1994; 74:474
Abnormal Immune Response?
Maternal immunologic response to a fetal
antigen?
  Fetal cells may escape into the maternal
  circulation without being rejected.
  May become lodged in cardiac tissue.
  May trigger immune response


   *Nelson et al. J Am Med Womens Assoc 1998; 53:31
 Immune Response? Cont….
Disproved 1990., Nigerian Study
  39 women with PPCM
  No differences between subjects and controls
  in levels of:
     Serum Immunoglobulins
     Circulating Immune Complexes
     Cardiac muscle antibodies

   *Cenac et al. Int J Cardiol 1990; 26:49
                     Genetics
Several case reports published
  1963, Pierce et al. reported that 3 of 17 patients with PPCM had
  definitive FH of same condition
  1984 Voss et al. reported a patient who died from PPCM as did her
  mother and two of her sisters
  1993 Massad et al. reported 16 y.o girl with PPCM following
  molar preg. Sister later received cardiac transplant for PPCM.



Cont….
                Genetics cont….
Also, 1976 Strung documented male relatives of
female patients with PPCM as also having
cardiomyopathies.

Hard to retrospectively study….
   Can not determine every patient who develops PPCM
   was completely healthy before pregnancy.

*Pearl Am Heart J 1995;129:421-2
             Risk Factors
Age >30 years old     Pregnancy with
Multiparity           multiple fetuses
African Descent       History of
Maternal cocaine      Preeclampsia,
abuse                 eclampsia, or
                      postpartum HTN
Long term tocolytic
therapy (>4weeks)
      Criteria for Diagnosis
4 Criteria
  Development of Heart failure in the last month
  of pregnancy, or within 5 months postpartum
  Absence of a determinable cause for cardiac
  failure
  Absence of heart disease before last month of
  pregnancy
  Left Ventricle impairment demonstrated on
  Echo
       Clinical Presentation
Symptoms:                Signs:
  Paroxysmal Nocturnal     Cardiomegaly
  Dyspnea                  Gallop Rhythm
  Dyspnea on Exertion      Edema
  Cough                    Holosystolic murmur
  Orthopnea
  Chest Pain
  Abdominal Discomfort
  Palpitation
                               ns in
                            tio l Pa
PPCM Symptoms




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                     Pa
                90
                80
                70
                60
                50
                40
                30
                20
                10
                 0
       Timing of Diagnosis
Dx. Requires being in
the last month of
pregnancy
If earlier, consider
underlying heart
disease (ischemic,
valvular, or
myopathic)
 2nd trimester burden
               Diagnosis
EKG
Two-dimensional echocardiogram
CXR
Lab: CBC, CMP, BNP, TSH, Ferritin
If persistent past initial therapy:
  Cardiac catheterization
  ?Myocardial biopsy
           EKG Changes
Sinus Tachycardia
Nonspecific ST
changes
LV Hypertrophy
             Chest X-ray
Pulmonary Edema
Venous congestion
Enlarged Cardiac
Silhouette
R/O PE
          Echocardiogram
Spherical LV
Mitral and Tricuspid
regurgitation
Left Atrial
enlargement
EF <55%
         Case Presentation
EKG WNL
CXR-mild edema
Echo:
  EF 47%
  Mild Mitral Regurg
  Mild LV dilatation
  Mild LV hypokinesis
  Mild LA dilatation
                     Treatment
 Delivery
 Similar to other forms of CHF
    Diuretics
    ß-blockers
    Digoxin
    Anticoagulants

*Must consider pregnancy class/breast-feeding
 harm potential!
Pregnancy Drug Class Review
Category A: Controlled studies in pregnant women
fail to demonstrate a risk to the fetus in the first
trimester with no evidence of risk in later trimesters.
The possibility of harm appears remote
Category B: Presumed safety based on animal
studies, with no controlled studies in pregnant
women, or animal studies have shown an adverse
effect that was not confirmed in controlled studies in
women in the first trimester and there is no evidence
of a risk in later trimesters.
          Drug class cont…..
Category C: Studies in women and animals are not
available or studies in animals have revealed
adverse effects on the fetus and there are no
controlled studies in women. Drugs should be given
only if the potential benefits justify the potential risk to
the fetus
Category D: There is positive evidence of human
fetal risk (unsafe), however in some cases such as a
life-threatening illness the potential risk may be
justified if there are no other alternatives
       Drug class cont….
Category X: Highly unsafe: risk of use
outweighs any potential benefit. Drugs
in this category are contraindicated in
women who are or may become
pregnant
                     Drugs
Digoxin Class C
  Symptomatic control
  Requires level
  monitoring
  Therapeutic levels 0.7-
  1.2
                 Diuretics
Lasix Class C              Thiazide Diuretics
  Reserved for cardiac       Reserved for cardiac
  conditions                 conditions
  Not recommended in         Not recommended in
  PIH                        PIH
  May decrease placental     Thrombocytopenia has
  perfusion                  been reported in breast
                             feeding infants
              Vasodilators
Hydralazine Class C   ACE Inhibitors
  Compatible with       Class D in 2nd/3rd
  breastfeeding         trimesters
                        Reserved for
                        postpartum use-
                        compatible with BF
                        Renal toxicity in
                        infants exposed in
                        utero
          Beta-Blockers
Class C
Compatible with breast feeding
Has been shown to cause IUGR in some
infants in utero.
             Anticoagulants
Heparin Class C            Warfarin Class D
  Short half life-can be     Contraindicated in
  discontinued prior to      pregnancy
  delivery to prevent        Safe in breast feeding.
  maternal hemorrhage        Not excreted in breast
  Not excreted in breast     milk.
  milk
                 Other Therapy
IV Immune Globulin
  One retrospective study
       6 PPCM treated
       11 controls
       All 6 treated had >10 units improvement in EF,
       compared only 4/11 controls
  (All pts had diagnosis of Myocarditis and
  dilated cardiomyopathy)
*McNamara et al. Circulation 1997; 95:2476
       Other Therapy cont….
Cardiac Transplant
  Estimated that transplant is performed in up to
  1/3 of PPCM patients
  Pts should be strongly advised against future
  pregnancies.
     Increased risk of HTN, preeclampsia, and preterm
     labor
     Also at risk for graft failure due to recurrent disease.
   *Scott et al. Obstet Gynecol 1993; 82:324
      Differential Diagnosis
PIH
  However, HF associated with PIH represents a
  diastolic failure, vs. systolic in PPCM
Pulmonary Embolism
  Again, usually ruled out by CXR
  If still suspicious, can order spiral CT
                Prognosis
Mortality estimates range from 25-50%.
Most deaths occur within 3 months
postpartum
  Deaths usually caused by:
    Progressive pump failure
    Arrhythmias
    Thromboembolic events
             Prognosis cont…
India study
  20 pts. PPCM
  Followed for 14 months postpartum
  Found several factors for deterioration:
     Age >30
     High Parity
     Later onset of sx. Following pregnancy
     Worse echo findings on initial exam

   *Elkayam et al. N Engl J Med 2001; 344:1567
      Future Pregnancies??
Opinions widely vary
Most experts agree that patients should
avoid future pregnancy if LV dysfunction
is persistent greater than 6 months
                        Literature
One study:
  NEJM 2001 – USC
     44 Patients PPCM undergoing subsequent preg.
     28 had normal LV function
     16 had persistent LV dysfunction
     Results:
       –   Average 10% drop in LVEF in normalized group
       –   Average 4% drop in LVEF in dysfunctional group
       –   More than 20% drop in >21% of patients in group 1
       –   19% mortality rate in group 2

       *Elkayam et al. N Engl J Med 2001; 444:1567
  Future Pregnancies cont…
Highly Individual
  Patient education of risks
  MFM, Cardiology involvement in decision

If future pregnancy desired:
  Maternal Echocardiogram per trimester
  Serial sonograms for growth
  Again, Subspecialty involvement
                      Summary
PPCM –Dilated myopathy
1:3000-4000 pregnancies
Maternal mortality Increasing!
36 WGA- 5mo. Postpartum
Symptoms:
   Dyspnea, Edema, Orthopnea
EKG, CXR, Echocardiogram
   CBC, CMP, BNP, TSH, etc.
Tx: Diuretics, B-blockers,
ACEI, Anticoagulants
Consult, consult, consult
Prognosis varies
Future Pregnancies…..???
                     References
Demakis, JG, Rahimtoola, SH, Sutton, GC, et al. Natural course of
peripartum cardiomyopathy. Circulation 1971; 44:1053
Sanderson, JE, Olsen, EG, Gatei, D. Peripartum heart disease: An
endomyocardial biopsy study. Br Heart J 1986; 56:285
Midei, MG, DeMent, SH, Feldman, AM, et al. Peripartum myocarditis
and cardiomyopathy. Circulation 1990; 81:922
O'Connell, JB, Costanzo-Nordin, MR, Subramanian, R, et al.
Peripartum cardiomyopathy: Clinical, hemodynamic, histologic and
prognostic characteristics. J Am Coll Cardiol 1986; 8:52
Rizeq, MN, Rickenbacher, PR, Fowler, MB, et al. Incidence of
myocarditis in peripartum cardiomyopathy. Am J Cardiol 1994;
74:474
Nelson, JL. Pregnancy, persistent microchimerism, and autoimmune
disease. J Am Med Womens Assoc 1998; 53:31
Cenac, A, Beaufils, H, Soumana, I, et al. Absence of humoral
autoimmunity in peripartum cardiomyopathy. A comparative study in
Niger. Int J Cardiol 1990; 26:49
               References cont…
Pearl, W. Familial occurrence of peripartum cardiomyopathy. Am
Heart J 1995; 129:421
McNamara, DM, Rosenblum, WD, Janosko, KM, et al. Intravenous
immune globulin in the therapy of myocarditis and acute
cardiomyopathy. CIrculation 1997; 95:2476
Scott, JR, Wagoner, LE, Olsen, SL, et al. Pregnancy in heart
transplant recipients: management and outcome. Obstet Gynecol
1993; 82:324
Elkayam, U, Tummala, PP, Rao, K, et al. Maternal and fetal outcomes
of subsequent pregnancies in women with peripartum
cardiomyopathy. N Engl J Med 2001; 344:1567
Pearl,W. Familial Occurrence of peripartum Cardiomyopathy. Am Heart Journal 1995;
129:421-22
Sliwa, K, Forster, O, Zhanje, F, et al. Outcome of subsequent
pregnancy in patients with documented peripartum cardiomyopathy.
Am J Cardiol 2004; 93:1441

				
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