Peripartum Cardiomyopathy (PPCM)



What is PPCM?

• A form of acute systolic heart failure that develops late in pregnancy or within 5 months postpartum. (Can occur later than 5 mos too)

• Characterized by left ventricular ejection fraction <45%, without preexisting heart disease.



Epidemiology:

• Occurs in ~1 in 2000 births globally.

• Higher incidence in:

   - Haiti (1 in 300)

   - Nigeria (1 in 100)

• In the U.S., Black women are 4x more likely to develop PPCM than White women.



Risk Factors:

• Hypertensive disorders of pregnancy (e.g., preeclampsia)

• Multiple gestations

• Advanced maternal age

• Anemia

✗ NOT a risk factor: Cesarean delivery



Clinical Presentation:

• Mimics normal pregnancy symptoms resulting in delayed diagnosis:

   - Dyspnea, orthopnea, edema

• Can escalate to:

  ⚠️ Cardiogenic shock

  ⚠️ Arrhythmias

  ⚠️ Thromboembolic events

• Key Point: High clinical suspicion is needed to avoid diagnostic delays



Pathogenesis:

Peripartum cardiomyopathy (PPCM) is caused by a combination of hormonal changes and heart vulnerability in late pregnancy or early postpartum:



🧬 Hormonal Triggers

• Prolactin (cleaved form): damages blood vessels, harms heart cells

• sFlt-1 & Activin A (from placenta): block blood vessel growth and damage heart function

• Progesterone: impairs heart metabolism

• ↓ Relaxin: reduces vascular protection



🧬 Underlying Susceptibility

• Genetic variants (e.g., TTN): weaken heart muscle resilience

• Other possible factors: inflammation, autoimmunity, nutrient deficiencies (e.g., selenium)



Differential diagnosis:

• Preexisting structural heart disease

• Preeclampsia-induced pulmonary edema in the absence of systolic dysfunction

• Pulmonary/amniotic embolism

• Spontaneous coronary artery dissection

• Exposure to toxins, including alcohol and chemotherapeutic agents

• Valvular heart disease, including rheumatic disease

• Myocarditis, including giant-cell myocarditis

• Takotsubo cardiomyopathy

• Tachycardia-induced cardiomyopathy

• Pulmonary edema resulting from prolonged tocolysis

• Sepsis, thyrotoxicosis, and other high-output causes of heart failure

• Aortic dissection

• Other causes of myocardial infarction, including MINOCA



Diagnosis:

• Echocardiography: LVEF <45%

• Left ventricular dilatation is common but not always seen

• Rule out other causes of heart failure

• Consider biomarkers (e.g., BNP) and imaging

• Nonspecific: sinus tachycardia on the electrocardiogram and pulmonary venous congestion on a CXR



Outcomes & Prognosis:

• 50% of patients experience recovery of cardiac function

• However:

   - Up to 20% mortality

   - Higher in Black women and in resource-limited settings

   - Some patients require LVAD or heart transplantation



Key Takeaways:

• PPCM is underdiagnosed due to symptom overlap with pregnancy.

• Early recognition and management can significantly improve outcomes.

• Health disparities impact incidence, recovery, and mortality.

• The neurohormonal changes of late gestation and parturition probably trigger peripartum cardiomyopathy in genetically or otherwise susceptible women



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