Peripartum Cardiomyopathy Matthew Voth M.D. WCGME Dept. of Ob/Gyn – PGY-1
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
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.
Tight nuchal cord
Pt. To recovery in
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
PIH labs ordered - WNL
Postpartum Care cont….
PPD #4, Hgb 7.4
BP 138/85, pt. Asymptomatic
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
Pulse 83, regular
O2 sat 100% on 1L
2+ edema LE’sL
Lungs crackles heard at bases bilaterally
PIH labs ordered
20 mg Lasix given in ER
Admitted to 3-WH
Dx: R/O cardiomyopathy
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
Post admit day 1- pt reportedly much improved. Breathing easier. Ambulating. Voiding >90cc/hour.
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 4 th 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
Increase in TBV due to:
Increased vascular capacitance
… .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
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
Overall responsible for 10,000 deaths and 46,000 hospitalizations each year
Wide age range 20-60
*Dec et al. N Engl J Med 1994; 331:1564
Progressive dyspnea with exertion
Impaired exercise capacity
Paroxysmal nocturnal dyspnea
*Felker et al. N Engl J Med 2000; 342:1077
4% of all cardiomyopathies
Should we be concerned??
CDC Pregnancy Related Mortality Surveillance 1991-1999
Leading Causes of Maternal Mortality:
Embolism – 20%
Hemorrhage – 17%
Hypertension – 16%
Peripartum Cardiomyopathy- 9%***
Multiple studies have attempted to elucidate a distinct etiology…..all have failed
Abnormal Immune Response
High postpartum salt intake
11 African women with PPCM
Endocardial biopsies done on all eleven
5 showed evidence of “healing myocarditis”
Presence of inflammatory cells
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
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
3 rd 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
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:
Circulating Immune Complexes
Cardiac muscle antibodies
*Cenac et al. Int J Cardiol 1990; 26:49
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.
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
Age >30 years old
Maternal cocaine abuse
Long term tocolytic therapy (>4weeks)
Pregnancy with multiple fetuses
History of Preeclampsia, eclampsia, or postpartum HTN
Criteria for Diagnosis
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
Paroxysmal Nocturnal Dyspnea
Dyspnea on Exertion
Timing of Diagnosis
Dx. Requires being in the last month of pregnancy
If earlier, consider underlying heart disease (ischemic, valvular, or myopathic)
2 nd trimester burden
Lab: CBC, CMP, BNP, TSH, Ferritin
If persistent past initial therapy:
Nonspecific ST changes
Enlarged Cardiac Silhouette
Mitral and Tricuspid regurgitation
Left Atrial enlargement
Mild Mitral Regurg
Mild LV dilatation
Mild LV hypokinesis
Mild LA dilatation
Similar to other forms of CHF
* 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
Digoxin Class C
Requires level monitoring
Therapeutic levels 0.7-1.2
Lasix Class C
Reserved for cardiac conditions
Not recommended in PIH
May decrease placental perfusion
Reserved for cardiac conditions
Not recommended in PIH
Thrombocytopenia has been reported in breast feeding infants
Hydralazine Class C
Compatible with breastfeeding
Class D in 2 nd /3 rd trimesters
Reserved for postpartum use-compatible with BF
Renal toxicity in infants exposed in utero
Compatible with breast feeding
Has been shown to cause IUGR in some infants in utero.
Heparin Class C
Short half life-can be discontinued prior to delivery to prevent maternal hemorrhage
Not excreted in breast milk
Warfarin Class D
Contraindicated in pregnancy
Safe in breast feeding. Not excreted in breast milk.
IV Immune Globulin
One retrospective study
6 PPCM treated
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….
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
However, HF associated with PIH represents a diastolic failure, vs. systolic in PPCM
Again, usually ruled out by CXR
If still suspicious, can order spiral CT
Mortality estimates range from 25-50%.
Most deaths occur within 3 months postpartum
Deaths usually caused by:
Progressive pump failure
20 pts. PPCM
Followed for 14 months postpartum
Found several factors for deterioration:
Later onset of sx. Following pregnancy
Worse echo findings on initial exam
* Elkayam et al. N Engl J Med 2001; 344:1567
Opinions widely vary
Most experts agree that patients should avoid future pregnancy if LV dysfunction is persistent greater than 6 months
NEJM 2001 – USC
44 Patients PPCM undergoing subsequent preg.
28 had normal LV function
16 had persistent LV dysfunction
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…
Patient education of risks
MFM, Cardiology involvement in decision
If future pregnancy desired:
Maternal Echocardiogram per trimester
Serial sonograms for growth
Again, Subspecialty involvement
PPCM –Dilated myopathy
Maternal mortality Increasing!
36 WGA- 5mo. Postpartum
Dyspnea, Edema, Orthopnea
EKG, CXR, Echocardiogram
CBC, CMP, BNP, TSH, etc.
Tx: Diuretics, B-blockers, ACEI, Anticoagulants
Consult, consult, consult
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
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