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Peripartum Cardiomyopathy

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Title: Peripartum Cardiomyopathy


1
Peripartum Cardiomyopathy
  • Matthew Voth M.D.
  • WCGME
  • Dept. of Ob/Gyn PGY-1

2
Case Presentation
  • N.A. 22 y.o. G1 P0 _at_ 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

3
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

4
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.

5
Emergent C/S
  • No complications
  • EBL 1000cc
  • APGARS 8/9
  • Tight nuchal cord
  • Pt. To recovery in
  • stable condition

6
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

7
Postpartum Day 2
  • A.M. Hgb 7.4 gm/dl
  • Pt. Not tachycardic, BPs stable 130s/70s
  • Urine output 100cc/hour
  • IV DCd PPD 2

8
Postpartum Care cont…
  • Pt. Remained asymptomatic.
  • Vital signs remained stable until PPD3
  • 4 consecutive BPs 140/90 and HR 110
  • Pt. Tol PO well. IV not restarted
  • C/O Headache
  • PIH labs ordered - WNL

9
Postpartum Care cont….
  • PPD 4, Hgb 7.4
  • BP 138/85, pt. Asymptomatic
  • Discharged home

10
ER Visit PPD7
  • 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

11
Physical Exam
  • BP 143/100
  • Pulse 83, regular
  • RR 19
  • O2 sat 100 on 1L
  • 2 edema LEsL
  • 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

12
Cardiology consult
  • EKG- normal
  • BMP WNL
  • CBC Hgb 8.1 gm/dl
  • TSH - WNL
  • Troponin I WNL
  • BNP 949 normal range (
  • Echo Dilated cardiomyopathy

13
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 Is and Os

14
3-Womens
  • 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.

15
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 98393

16
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 3191127

17
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 256H1060.

18
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19
Chapman et al. Kidney Int 1998 542056
20
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 93841

21
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 3311564
  • Common Sx
  • Progressive dyspnea with exertion
  • Impaired exercise capacity
  • Orthopnea
  • Paroxysmal nocturnal dyspnea
  • Peripheral edema

22
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23
Felker et al. N Engl J Med 2000 3421077
24
Peripartum Cardiomyopathy
  • 4 of all cardiomyopathies
  • 13000-4000 preg.
  • Dilated Cardiomyopathy

25
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

26
Etiology
  • Multiple studies have attempted to elucidate a
    distinct etiology…..all have failed
  • Theories
  • Myocarditis
  • Abnormal Immune Response
  • Genetics
  • High postpartum salt intake

27
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 56285

28
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
  • OConnell et al. J AM Coll Cardiol 1986 852

29
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 81922

30
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 74474

31
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 5331

32
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 2649

33
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….

34
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 1995129421-2

35
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

36
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

37
Clinical Presentation
  • Symptoms
  • Paroxysmal Nocturnal Dyspnea
  • Dyspnea on Exertion
  • Cough
  • Orthopnea
  • Chest Pain
  • Abdominal Discomfort
  • Palpitation
  • Signs
  • Cardiomegaly
  • Gallop Rhythm
  • Edema
  • Holosystolic murmur

38
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39
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

40
Diagnosis
  • EKG
  • Two-dimensional echocardiogram
  • CXR
  • Lab CBC, CMP, BNP, TSH, Ferritin
  • If persistent past initial therapy
  • Cardiac catheterization
  • ?Myocardial biopsy

41
EKG Changes
  • Sinus Tachycardia
  • Nonspecific ST changes
  • LV Hypertrophy

42
Chest X-ray
  • Pulmonary Edema
  • Venous congestion
  • Enlarged Cardiac Silhouette
  • R/O PE

43
Echocardiogram
  • Spherical LV
  • Mitral and Tricuspid regurgitation
  • Left Atrial enlargement
  • EF

44
Case Presentation
  • EKG WNL
  • CXR-mild edema
  • Echo
  • EF 47
  • Mild Mitral Regurg
  • Mild LV dilatation
  • Mild LV hypokinesis
  • Mild LA dilatation

45
Treatment
  • Delivery
  • Similar to other forms of CHF
  • Diuretics
  • ß-blockers
  • Digoxin
  • Anticoagulants
  • Must consider pregnancy class/breast-feeding
    harm potential!

46
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.

47
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

48
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

49
Drugs
  • Digoxin Class C
  • Symptomatic control
  • Requires level monitoring
  • Therapeutic levels 0.7-1.2

50
Diuretics
  • Lasix Class C
  • Reserved for cardiac conditions
  • Not recommended in PIH
  • May decrease placental perfusion
  • Thiazide Diuretics
  • Reserved for cardiac conditions
  • Not recommended in PIH
  • Thrombocytopenia has been reported in breast
    feeding infants

51
Vasodilators
  • Hydralazine Class C
  • Compatible with breastfeeding
  • ACE Inhibitors
  • Class D in 2nd/3rd trimesters
  • Reserved for postpartum use-compatible with BF
  • Renal toxicity in infants exposed in utero

52
Beta-Blockers
  • Class C
  • Compatible with breast feeding
  • Has been shown to cause IUGR in some infants in
    utero.

53
Anticoagulants
  • 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.

54
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 952476

55
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 82324

56
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

57
Prognosis
  • Mortality estimates range from 25-50.
  • Most deaths occur within 3 months postpartum
  • Deaths usually caused by
  • Progressive pump failure
  • Arrhythmias
  • Thromboembolic events

58
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 3441567

59
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60
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61
Future Pregnancies??
  • Opinions widely vary
  • Most experts agree that patients should avoid
    future pregnancy if LV dysfunction is persistent
    greater than 6 months

62
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 4441567

63
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

64
Summary
  • PPCM Dilated myopathy
  • 13000-4000 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…..???

65
References
  • Demakis, JG, Rahimtoola, SH, Sutton, GC, et al.
    Natural course of peripartum cardiomyopathy.
    Circulation 1971 441053
  • Sanderson, JE, Olsen, EG, Gatei, D. Peripartum
    heart disease An endomyocardial biopsy study. Br
    Heart J 1986 56285
  • Midei, MG, DeMent, SH, Feldman, AM, et al.
    Peripartum myocarditis and cardiomyopathy.
    Circulation 1990 81922
  • O'Connell, JB, Costanzo-Nordin, MR, Subramanian,
    R, et al. Peripartum cardiomyopathy Clinical,
    hemodynamic, histologic and prognostic
    characteristics. J Am Coll Cardiol 1986 852
  • Rizeq, MN, Rickenbacher, PR, Fowler, MB, et al.
    Incidence of myocarditis in peripartum
    cardiomyopathy. Am J Cardiol 1994 74474
  • Nelson, JL. Pregnancy, persistent microchimerism,
    and autoimmune disease. J Am Med Womens Assoc
    1998 5331
  • Cenac, A, Beaufils, H, Soumana, I, et al. Absence
    of humoral autoimmunity in peripartum
    cardiomyopathy. A comparative study in Niger. Int
    J Cardiol 1990 2649

66
References cont…
  • Pearl, W. Familial occurrence of peripartum
    cardiomyopathy. Am Heart J 1995 129421
  • McNamara, DM, Rosenblum, WD, Janosko, KM, et al.
    Intravenous immune globulin in the therapy of
    myocarditis and acute cardiomyopathy. CIrculation
    1997 952476
  • Scott, JR, Wagoner, LE, Olsen, SL, et al.
    Pregnancy in heart transplant recipients
    management and outcome. Obstet Gynecol 1993
    82324
  • 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 3441567
  • Pearl,W. Familial Occurrence of peripartum
    Cardiomyopathy. Am Heart Journal 1995 129421-22
  • Sliwa, K, Forster, O, Zhanje, F, et al. Outcome
    of subsequent pregnancy in patients with
    documented peripartum cardiomyopathy. Am J
    Cardiol 2004 931441
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