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
• Identifying Clinical Features
• Appropriate Tests
• Differential Diagnosis
• Management Considerations
• Pregnancy Specific Considerations
• Subsequent Pregnancies

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
  • Normal pregnancy may increase BNP or NT-proBNP up to twofold while values remain within the normal range
  • Interpret with caution in
    • Obesity | Preeclampsia | Congenital heart disease | Cardiomyopathy | Sepsis | Renal failure | Pulmonary embolism | Critical illness | Anemia
• 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 and not in acute decompensated left ventricular heart failure
  • Selective β1 receptor blocker | Metoprolol preferred to avoid uterine stimulation via β2 pathway | Avoid beta-blockers in acute decompensated left ventricular heart failure
• 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
• Use anticoagulation if any of the following apply
  • LVEF <35%
  • Acute left ventricular heart failure during hospitalization
  • Bromocriptine use
• Cardioversion and defibrillation may be used in emergent settings (safe in pregnancy)
• Bromocriptine therapy
  • Sympatholytic dopamine D2 agonist
  • Investigational and under study | Limited human data suggest possible benefit in left ventricular recovery | Not confirmed in larger trials
  • 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 maternal 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
• Prenatal ultrasound
  • Serial growth ultrasounds during pregnancy
  • Fetal echocardiography when maternal heart failure is due to underlying congenital cardiac disease
• Timing of delivery
  • Stable: Per obstetric indications
  • Unstable or maternal extremis: Prompt delivery
• Mode of delivery
  • Vaginal delivery at term preferred is patient is stable
• Anesthesia
  • Neuraxial analgesia and anesthesia are considered safe
  • Benefits: Reduces cardiac output fluctuation due to catecholamines | Patient is prepared in case of rapid or emergency cesarean
• Breastfeeding
  • Routine counseling recommended | Review each medication for compatibility | Use shared decision making | ACE inhibitors are generally compatible with breastfeeding | Avoid DOACs while breastfeeding

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
  • Ensure contraception counseling prior to discharge | Progestin-releasing IUDs are the preferred contraception option for women with high-risk cardiac conditions
• Women with normal function prior to subsequent pregnancy are still at increased risk (20%) of worsening cardiac function

Learn More – Primary Sources

SMFM Consult Series 73: Diagnosis and management of right and left heart failure during pregnancy and postpartum

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

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

2022 AHA/ACC/HFSA: Guideline for the Management of Heart Failure

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

Alliance for Innovation on Maternal Health: Consensus Bundle on Cardiac Conditions in Obstetrics

BJOG Review: Cardiogenic shock in pregnancy

Contemporary Management of Cardiomyopathy and Heart Failure in Pregnancy

Peripartum cardiomyopathy: a comprehensive and contemporary review

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