🤰❤️ Peripartum Cardiomyopathy (PPCM) is a rare, life-threatening cause of heart failure occurring in late pregnancy or the early postpartum period (last month of pregnancy up to 5 months postpartum).
High clinical suspicion is essential because symptoms often overlap with normal pregnancy.
Prompt diagnosis, guideline-directed management, and long-term follow-up improve outcomes and reduce recurrence risk.
⚡ Key Points
- Consider in any pregnant/postpartum woman with unexplained dyspnoea, orthopnoea, fatigue, or peripheral oedema.
- Always exclude other causes: pulmonary embolism, pneumonia, preeclampsia/eclampsia, valvular or ischaemic heart disease.
- Early stabilisation: ABCs → oxygen, cautious diuretics, continuous monitoring, involve obstetrics and cardiology.
- High risk of thromboembolism → anticoagulation may be indicated (IV unfractionated heparin in pregnancy; LMWH or warfarin postpartum).
🧬 Pathophysiology
- Exact mechanism unknown; multifactorial: oxidative stress → cleavage of prolactin to 16 kDa fragment → anti-angiogenic, pro-apoptotic effects on myocardium.
- Inflammatory cytokines, vascular dysfunction, genetic susceptibility (TTN mutations) contribute.
- Results in dilated cardiomyopathy phenotype with left ventricular systolic dysfunction and sometimes LV thrombus.
⚠️ Risk Factors
- Maternal age ≥30 years
- Multiparity 👩👧👦
- African or African-Caribbean ethnicity 🌍
- Multiple gestation (twins/triplets)
- Preeclampsia, eclampsia, postpartum hypertension
- Use of cocaine or long-term oral β-agonists (tocolytics)
- Previous PPCM in prior pregnancy → very high recurrence risk
🩺 Clinical Features
- Dyspnoea on exertion, orthopnoea, paroxysmal nocturnal dyspnoea
- Fatigue, reduced exercise tolerance
- Peripheral oedema, raised JVP, hepatomegaly
- Palpitations, arrhythmias, chest discomfort
- Thromboembolic events: stroke, systemic emboli (esp. if EF <35%)
🔎 Investigations
- Bloods: FBC, U&E, LFTs, CRP, BNP/NT-proBNP (↑ in HF), ± troponin
- ECG: Non-specific; may show sinus tachycardia, arrhythmias, conduction abnormalities
- CXR: Cardiomegaly, pulmonary congestion, pleural effusions (safe with shielding)
- Echocardiography: Diagnostic; LV systolic dysfunction (EF <45%), ± LV dilatation, RV involvement, thrombus
- Cardiac MRI: Tissue characterisation; exclude myocarditis
- Coronary angiography: Exclude ischaemic heart disease in older or high-risk patients
💊 Management (NICE/ESC Compliant)
- Acute stabilisation: ABCs, oxygen, cautious IV diuretics, high-dependency or ICU monitoring
- Heart failure therapy:
- Beta-blockers: labetalol, metoprolol – safe in pregnancy
- Vasodilators: hydralazine + nitrates for afterload reduction (avoid ACEI/ARBs antenatally)
- Diuretics: judicious to reduce pulmonary congestion; avoid reducing placental perfusion
- Avoid ACE inhibitors, ARBs, mineralocorticoid antagonists during pregnancy (teratogenic)
- Anticoagulation: indicated if EF <35% or LV thrombus; IV unfractionated heparin preferred antenatally; LMWH or warfarin postpartum (warfarin contraindicated during pregnancy)
- Advanced therapies: Inotropes, mechanical support (IABP, LVAD), cardiac transplantation in refractory cases
- Delivery planning: Vaginal delivery preferred if maternal haemodynamics stable; Caesarean for obstetric indications; tertiary centre with multidisciplinary input
- Postpartum care & counselling: Contraception advice; high recurrence risk in future pregnancies if EF not fully recovered; long-term cardiology follow-up
📉 Prognosis
- Partial or complete LV recovery in 50–70% within 6–12 months
- Persistent LV dysfunction → high morbidity, mortality, and recurrence risk
- Mortality: 10–20%, higher in severe LV dysfunction or delayed diagnosis
🩺 Student Case Examples
Case 1 – Postpartum Dyspnoea: 30F, 3 weeks postpartum, progressive breathlessness, orthopnoea, ankle swelling. Exam: raised JVP, basal crackles, oedema. Echo: LVEF 25%.
Management: Diuretics, beta-blocker, ACEI/ARB postpartum, anticoagulation if EF <35%, cardiology follow-up. Avoid ACEI/ARBs during pregnancy.
Case 2 – Late Pregnancy Presentation: 36F, 36 weeks’ gestation, fatigue, palpitations, nocturnal breathlessness. Exam: tachycardia, displaced apex, basal crepitations. BNP ↑, Echo EF 30%.
Management: Pregnancy-safe HF therapy: loop diuretics, beta-blocker, hydralazine + nitrates. Plan delivery in tertiary centre with obstetric and cardiology input. Avoid ACEI/ARBs and mineralocorticoid antagonists.
📚 References