How is peripartum cardiomyopathy diagnosed and managed in a previously healthy woman aged 20‑40 presenting in the third trimester or early postpartum period with dyspnea, orthopnea, peripheral edema and left‑ventricular ejection fraction ≤45%?

Peripartum Cardiomyopathy: Diagnosis and Management

Diagnostic Approach

Peripartum cardiomyopathy is a diagnosis of exclusion requiring echocardiographic confirmation of left ventricular ejection fraction below 45% in a woman presenting with heart failure symptoms in late pregnancy or the postpartum period, after ruling out other cardiac pathology. 1

Clinical Presentation Recognition

• The majority of patients (78%) present within the first 4 months postpartum, with peak incidence occurring 2-62 days after delivery. 2, 3
• Only 9% present during the last month of pregnancy, making third-trimester presentation less common but still within the diagnostic window. 2
• Typical symptoms include dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea, marked peripheral edema, persistent cough, and abdominal discomfort from hepatic congestion. 1, 3
• Physical examination findings include displaced apical impulse (72%), third heart sound (92%), and mitral regurgitation (43%). 1

Critical Diagnostic Pitfall: Distinguishing from Pre-eclampsia

• Pre-eclampsia with pulmonary edema is the most important differential diagnosis, as it requires fundamentally different management (aggressive blood pressure control versus standard heart failure therapy). 2
• The postpartum peak for PPCM (2-62 days after delivery) occurs beyond the typical window for pre-eclampsia-related complications. 2
• Severe hypertension requires exclusion of pre-existing hypertension in patients presenting before delivery. 2
• Studies that included higher proportions of pre-eclampsia patients showed more antepartum presentations, creating diagnostic confusion. 2

Essential Diagnostic Workup

Immediate echocardiography is mandatory to confirm:

• Left ventricular ejection fraction (nearly always <45% in PPCM). 1
• Left ventricular end-diastolic dimension. 4
• Presence of left ventricular thrombus (occurs in patients with LVEF <35%). 1, 4
• Right ventricular involvement (affects prognosis). 3

Additional required investigations:

• Electrocardiogram: Rarely normal in PPCM; 96% show ST-T wave abnormalities and 66% have voltage criteria for left ventricular hypertrophy. 1
• BNP or NT-proBNP: All PPCM patients have elevated levels due to increased left ventricular end-diastolic pressure. 1
• Comprehensive evaluation to exclude other causes: coronary artery disease, valvular heart disease, congenital heart disease, hypertensive heart disease, and pulmonary embolism. 1, 4

Management Strategy

Acute Phase Management

Initiate standard heart failure therapy immediately, modified for pregnancy/breastfeeding safety:

• Diuretics for volume overload and symptom relief. 4
• Beta-blockers for neurohormonal blockade. 4
• ACE inhibitors postpartum (contraindicated during pregnancy; use hydralazine-nitrate combination if still pregnant). 4

Anticoagulation Decision

Anticoagulation is mandatory if LVEF <35% due to 16-17% incidence of intracardiac thrombi and high thromboembolic risk. 3, 4

• Continue anticoagulation until 6-8 weeks postpartum at minimum. 3
• Left ventricular thrombosis is not uncommon in PPCM, with reported peripheral embolic episodes including cerebral, coronary, and mesenteric embolism. 1

Prognostic Stratification

Poor prognostic indicators requiring closer monitoring and consideration of advanced therapies:

• LVEF <30% at presentation. 3, 4
• Left ventricular end-diastolic diameter >60 mm. 4
• Presence of left ventricular thrombus. 3
• Right ventricular involvement. 3

Favorable prognosis indicators:

• In developed countries, 93% transplant/LVAD-free 1-year survival is achievable with appropriate management. 3
• Spontaneous and complete recovery of ventricular function can occur, which is unusual in other cardiomyopathies. 1

Follow-up Schedule

Repeat echocardiography at:

• Before hospital discharge. 4
• 6 weeks postpartum. 4
• 6 months postpartum. 4
• Annually thereafter. 4

Future Pregnancy Counseling

Subsequent pregnancies carry substantial risk and require careful counseling:

• Strongly contraindicate future pregnancy if LVEF <25% at diagnosis or if LVEF has not normalized. 4
• Women with persistent left ventricular dysfunction face 48% risk of further LVEF deterioration, 49% risk of heart failure symptoms, and 16% mortality in subsequent pregnancies. 3
• Even with recovered left ventricular function, subsequent pregnancy carries 27% risk of LVEF deterioration and 32% risk of heart failure symptoms. 3

Contraception recommendations:

• Intrauterine devices and progesterone-only contraception are preferred methods. 4
• Avoid combined hormonal contraceptives due to increased thromboembolism risk. 4
• Avoid barrier methods alone due to high failure rate. 4

Key Clinical Pitfalls to Avoid

• Do not dismiss symptoms as normal pregnancy-related fatigue or postpartum tiredness—maintain high clinical suspicion, especially in the first 2-3 months postpartum. 1, 2
• Do not confuse PPCM with pre-eclampsia—the management differs fundamentally, and misdiagnosis leads to inappropriate treatment. 2
• Do not delay echocardiography—rapid progression to end-stage heart failure can occur within days. 1
• Do not overlook anticoagulation in patients with LVEF <35%—thromboembolic complications are common and potentially fatal. 1, 3