peripartum cardiomyopathy
TRANSCRIPT
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
EBL 1000cc
APGARS 8/9
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
Pulse 83, regular
RR 19
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
Cardiology consulted
Dx: R/O 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 10,000 deaths and 46,000 hospitalizations each year
Wide age range 20-60
*Dec et al. N Engl J Med 1994; 331:1564
Common Sx:Progressive dyspnea with exertion
Impaired exercise capacity
Orthopnea
Paroxysmal nocturnal dyspnea
Peripheral edema
Causes of Cardiomyopathies
50
95 4 4 4 3 3 1
10
0
1020
30
4050
60
*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 Study198611 African women with PPCMEndocardial biopsies done on all eleven
5 showed evidence of “healing myocarditis”– Presence of inflammatory cells– Necrosis– Fibrous remodeling
9 patients finished study75% of myocarditis group developed persistent heart failure80% 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 PPCM29% 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 PPCM14 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 study34 patients diagnosed with PPCMResearches 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 Study39 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 published1963, 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
Multiparity
African Descent
Maternal cocaine abuse
Long term tocolytic therapy (>4weeks)
Pregnancy with multiple fetuses
History of Preeclampsia, eclampsia, or postpartum HTN
Criteria for Diagnosis
4 CriteriaDevelopment of Heart failure in the last month of pregnancy, or within 5 months postpartumAbsence of a determinable cause for cardiac failureAbsence of heart disease before last month of pregnancyLeft Ventricle impairment demonstrated on Echo
Clinical Presentation
Symptoms:Paroxysmal Nocturnal Dyspnea
Dyspnea on Exertion
Cough
Orthopnea
Chest Pain
Abdominal Discomfort
Palpitation
Signs:Cardiomegaly
Gallop Rhythm
Edema
Holosystolic murmur
0102030405060708090
PPCM Symptoms
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
Diureticsß-blockersDigoxinAnticoagulants
*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 CSymptomatic control
Requires level monitoring
Therapeutic levels 0.7-1.2
Diuretics
Lasix Class CReserved for cardiac conditions
Not recommended in PIH
May decrease placental perfusion
Thiazide DiureticsReserved for cardiac conditions
Not recommended in PIH
Thrombocytopenia has been reported in breast feeding infants
Vasodilators
Hydralazine Class CCompatible with breastfeeding
ACE InhibitorsClass D in 2nd/3rd 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 CShort half life-can be discontinued prior to delivery to prevent maternal hemorrhage
Not excreted in breast milk
Warfarin Class DContraindicated in pregnancy
Safe in breast feeding. Not excreted in breast milk.
Other Therapy
IV Immune GlobulinOne 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 TransplantEstimated that transplant is performed in up to 1/3 of PPCM patientsPts should be strongly advised against future pregnancies.
Increased risk of HTN, preeclampsia, and preterm laborAlso at risk for graft failure due to recurrent disease.
*Scott et al. Obstet Gynecol 1993; 82:324
Differential Diagnosis
PIHHowever, HF associated with PIH represents a diastolic failure, vs. systolic in PPCM
Pulmonary EmbolismAgain, 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 study20 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 function16 had persistent LV dysfunctionResults:
– 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 IndividualPatient education of risksMFM, Cardiology involvement in decision
If future pregnancy desired:Maternal Echocardiogram per trimesterSerial sonograms for growthAgain, Subspecialty involvement
SummaryPPCM –Dilated myopathy
1:3000-4000 pregnancies
Maternal mortality Increasing!
36 WGA- 5mo. Postpartum
Symptoms: Dyspnea, Edema, Orthopnea
EKG, CXR, EchocardiogramCBC, CMP, BNP, TSH, etc.
Tx: Diuretics, B-blockers, ACEI, Anticoagulants
Consult, consult, consult
Prognosis varies
Future Pregnancies…..???
ReferencesDemakis, 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; 129:421-22Sliwa, K, Forster, O, Zhanje, F, et al. Outcome of subsequent pregnancy in patients with documented peripartum cardiomyopathy. Am J Cardiol 2004; 93:1441