Peripartum Cardiomyopathy: Definitions, Diagnosis and Management

SUMMARY:

Peripartum cardiomyopathy (PPCM) is defined as heart failure that may develop toward the end of pregnancy or months after delivery without an identifiable cause. While prognosis has improved substantially over the past several years, women with peripartum cardiomyopathy are still at risk for adverse outcomes.

Table of Contents

Risk Factors

  • Maternal age ≥30 years | African ancestry | Hypertension | Anemia | Substance misuse | Asthma | Autoimmune disease | Preeclampsia or eclampsia | Multiple gestation | Obesity | Thyroid dysfunction | Prolonged tocolysis
  • Pathogenesis remains unknown but appears to be underlying background susceptibility with second ‘hit’ (e.g. endocrine factors of pregnancy)
  • Note: Preeclampsia and eclampsia are associated with PPCM and may have shared pathophysiology

Identifying Clinical Features

  • Pulmonary rales (left-sided congestion)
  • Elevated jugular venous pressure (right-sided congestion)
  • Symptoms of congestion
    • Dyspnea on exertion
    • Orthopnea
    • Paroxysmal nocturnal dyspnea
    • Lower extremity edema

Appropriate Tests

  • EKG: Sinus rhythm | Non-specific ST-segment or T-wave changes
  • Chest X-Ray: Pulmonary edema | Enlarged cardiac silhouette
  • B-type natriuretic peptide (BNP): Elevated
    • Note: Not elevated in normal pregnancy
  • Echocardiography is the most useful diagnostic tool
    • <45% LVEF (diagnostic requirement)
    • Right ventricular dilation (in some cases)
    • Pulmonary hypertension (in some cases)
    • Atrial enlargement (in some cases)
    • Atrioventricular valvular regurgitation (in some cases)

Differential Diagnosis

  • Benign dyspnea of pregnancy
    • Normal CXR | Normal echocardiogram
    • Treatment: No work up required
  • Asthma
    • Indicated by pulmonary function tests and bronchodilator response | Wheezing
    • Treatment: Bronchodilator therapy
  • Pulmonary embolism
    • Sudden onset |Tachycardia | Chest pain | Unremarkable pulmonary exam | DVT on LE imaging or PE on CT chest angiogram
    • Treatment: Anticoagulation
  • Amniotic fluid embolism
    • Sudden onset | Circulatory collapse (usually after labor) | Bleeding (from DIC) | Hypotension | Tachypnea | Crackles on exam
    • Treatment: Supportive care
  • Preeclampsia  
    • Hypertension | Proteinuria | Usually accompanied by neurologic symptoms (headache, dizziness) | Echocardiogram shows mildly decreased LVEF
    • Treatment: Proceed with delivery | Supportive care

SYNOPSIS:

Most women (50-80%) will make a full recovery (LVEF >50%) within first 6 months. For prognostic purposes, an LVEF ≥30% usually means a full recovery of left ventricular function is likely, while LVEF <30% suggests a slow or incomplete recovery with respect to achieving full ventricular function. Black ancestry is associated with reduced likelihood of recovery. Due to increased recognition and improved treatment, mortality has improved from 30-50% in 1970’s to 1.3-16% in 2000’s.

KEY POINTS:

Management Considerations

  • Early consultation with a cardiologist/ MFM
  • Sodium restriction may be required
  • Symptomatic pulmonary or peripheral edema present
    • Loop diuretic
  • If hemodynamics permit
    • Selective β1 receptor blocker: Metoprolol preferred to avoid uterine stimulation via β2 pathway
    • Avoid ACE inhibitors and angiotensin receptor blockers (ARBs) during pregnancy
      • Some of these medications may be used postpartum depending on lactation safety profile
  • Digoxin may be used in pregnancy
  • LVEF >35% or those on Bromocriptine (see below)
    • Use anticoagulation 
  • Cardioversion and defibrillation may be used in emergent settings (safe in pregnancy)
  • Bromocriptine therapy
    • Sympatholytic dopamine D2 agonist
    • Experimental only, based on evidence that prolactin may be involved in pathogenesis
    • Associated complications: Lactation and thromboembolic events
    • Consider on an individual basis in severe cases (LVEF <25%) pending larger trials

Pregnancy-Specific Considerations

  • Avoid over-diuresis to maintain perfusion of the placenta
  • Close monitoring throughout pregnancy and through 6-months post-partum with echocardiograms (clinical scenario may dictate alternate/ more frequent regimen)
    • Each trimester
    • Immediately after delivery
    • 4 weeks postpartum
  • Timing of delivery (AHA recommendations)
    • Stable: Per obstetric indications
    • Unstable or maternal extremis: Prompt delivery
  • Breastfeeding
    • Some controversy but unless severe LVEF, benefits may outweigh risks

Subsequent Pregnancies

  • LVEF prior to next pregnancy is the strongest predictor of outcome
  • If LVEF <50%
    • 50% risk of acute heart failure with worsening disease and increased mortality
    • Pregnancy contraindicated without recovery to normal LVEF thus “repeat pregnancy is contraindicated in women with PCCM”
    • Ensure contraception counseling prior to discharge
  • Women with normal function prior to subsequent pregnancy are still at increased risk (20%) of worsening cardiac function

Learn More – Primary Sources

BMJ: State of the Art Review: Peripartum cardiomyopathy (2019)

Peripartum Cardiomyopathy: JACC State-of-the-Art Review (2020)

Current Diagnostic and Treatment Strategies for Specific Dilated Cardiomyopathies: A Scientific Statement From the American Heart Association (2016)

Current management of patients with severe acute peripartum cardiomyopathy: practical guidance from the Heart Failure Association of the European Society of Cardiology Study Group on peripartum cardiomyopathy (2016)

2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy

‘Ten Commandments’ of the 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy

Cardiovascular Considerations in Caring for Pregnant Patients: A Scientific Statement From the American Heart Association

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