Updated: 2/23/2020

Peripartum Cardiomyopathy

Review Topic
  • Snapshot
    • A 34-year-old G4P3 woman of 37 weeks gestation presents to the hospital with progressive dyspnea and fatigue over 2 weeks duration. On physical exam, she is found to have bilateral lower extremity edema, bilateral basilar rales, and extra heart sound following S2 at the apex. She has jugular venous distension. A chest x-ray is done which confirms the diagnosis . She is started on supplemental oxygen and intravenous diuretics.
  • Introduction
    • Overview
      • peripartum cardiomyopathy (PPCM) is a rare, idiopathic complication of pregnancy that results in left ventricular systolic heart failure in women during the peripartum period
      • this is a potentially life-threatening condition that requires urgent evaluation, diagnosis, and intervention
        • treatment similar to other forms of heart failure
  • Epidemiology
    • Incidence
      • rare, 0.04-0.10% of live births
    • Demographics
      • onset between 36 weeks gestation and 5 months postpartum
      • usually occurs within 1 month postpartum
    • Risk factors
      • pre-eclampsia or eclampsia
      • pregnancy-induced or pre-existing hypertension
      • advanced maternal age
      • multiple gestation
      • high parity
      • african-american
    • Pathogenesis
      • mechanism
        • largely idiopathic, may be related to systemic angiogenic imbalance, elevated oxidative stress, and impaired VEGF signaling during pregnancy
  • Presentation
    • Symptoms
      • common symptoms
        • chest pain
        • heart failure symptoms
          • exertional dyspnea
          • cough
          • orthopnea
          • paroxysmal nocturnal dyspnea
    • Physical exam
      • bilateral lower extremity edema
      • jugular venous distension
      • displaced apical pulse
      • abdominal fullness
      • bilateral basilar crackles
      • S3 gallop
  • Imaging
    • Chest radiograph
      • findings
        • enlarged cardiac silhouette
        • pulmonary vascular congestion
        • Kerley-B lines
        • interstitial edema
    • Echocardiogram
      • indication
        • imaging study of choice in the diagnostic evaluation of PPCM
      • findings
        • decreased left ventricular ejection fraction
  • Studies
    • B-type natriuretic peptide (BNP)
      • usually elevated in PPCM, greater than in pregnant or postpartum women
    • Electrocardiogram (ECG)
      • findings
        • sinus tachycardia
        • nonspecific ST or T abnormalities
      • comments
        • as findings are nonspecific, ECG is generally done to distinguish PPCM from other conditions like pulmonary embolism
  • Differential
    • Pulmonary embolism
      • key distinguishing features
        • will present with dyspnea with no evidence of left ventricular dysfunction or enlarged cardiac silhouette on imaging
  • Treatment
    • Management approach
      • similar to management of heart failure except teratogenic medications (e.g., ACE-inhibitors, angiotensin receptor blockers (ARBs), aldosterone receptor antagonists) are avoided in pregnant women
    • Conservative
      • spontaneous vaginal delivery
        • preferred mode of delivery for hemodynamically stable patients
    • Medical
      • ACE-inhibitors or ARBs
        • indication
          • for postpartum patients or pregnant patients following delivery
      • hydralazine and nitrates
        • indication
          • for patients who cannot take ACE-inhibitors or ARBs
      • β-blockers
        • indication
          • for long term management in all patients
      • spironolactone or eplerenone
        • indication
          • treatment option for postpartum patients or pregnant patients following delivery
      • diuretics
        • indication
          • for symptomatic treatment of patients with evidence of pulmonary edema
    • Surgical
      • immediate delivery via cesarean section
        • indications
          • severe heart failure
          • hemodynamic instability refractory to treatment
  • Complications
    • Ventricular tachyarrhythmias
    • Chronic cardiomyopathy
      • occurs in women with baseline left ventricular dysfunction
  • Prognosis
    • More than 50% of patients recover within 6 months with medical treatment
    • 6-10% mortality
      • earlier diagnosis associated with improved survival

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