Peripartum Cardiomyopathy

Peripartum cardiomyopathy (PPCM) is idiopathic heart failure with left ventricular ejection fraction under 45%, presenting toward the end of pregnancy or in the months after delivery, in the absence of another cause.

Incidence ranges from 1 in 1000 to 1 in 4000 live births in the USA, 1 in 100 in Nigeria (Haiti 1 in 300).

Risk factors: African ancestry, age over 30, multiparity, multiple gestation, pre eclampsia, hypertension, prolonged tocolytic therapy, family history.

Pathophysiology: oxidative stress cleaves prolactin into a 16 kDa antiangiogenic fragment, causing vascular and myocardial damage; genetic (TTN) contribution in 15%.

Complications: cardiogenic shock, arrhythmia, thromboembolism, sudden death. Recovery of ejection fraction in 50 to 80% within 6 to 12 months; mortality 2 to 10%.

• Dyspnoea, orthopnoea, paroxysmal nocturnal dyspnoea, cough, oedema, fatigue, palpitations, often mistaken for normal late pregnancy symptoms.
• Tachycardia, raised jugular venous pressure, S3, pulmonary crackles, mitral regurgitation.
• Embolic events.

• Diagnosis of exclusion: onset in last month of pregnancy to 5 months postpartum, ejection fraction under 45%.
• NT proBNP elevated (below 100 pg/mL makes the diagnosis unlikely), troponin.
• ECG, echocardiography (dilated ventricle, thrombus), cardiac MRI (without gadolinium during pregnancy).
• Exclude pre existing cardiomyopathy, valvular disease, pulmonary embolism, amniotic fluid embolism, myocarditis, thyroid disease.
• Genetic testing if family history or poor recovery.

During pregnancy

• Diuretics (furosemide 20 to 80 mg), beta blocker (metoprolol succinate, bisoprolol), hydralazine 25 to 75 mg three times daily with isosorbide dinitrate or nitrates for afterload reduction. Avoid ACE inhibitors, ARBs, sacubitril, MRAs and SGLT2 inhibitors.
• Anticoagulation with enoxaparin 1 mg/kg twice daily when ejection fraction under 30 to 35%.
• Deliver by planned vaginal delivery with epidural when stable; caesarean for obstetric reasons or haemodynamic instability, ideally in a multidisciplinary centre.

Postpartum

• Full heart failure therapy: ACE inhibitor (enalapril 2.5 to 10 mg twice daily; safe in breastfeeding), beta blocker, spironolactone, diuretics, then sacubitril/valsartan and SGLT2 inhibitor when not breastfeeding.
• Bromocriptine 2.5 mg twice daily for 2 weeks then 2.5 mg daily for 6 weeks (suppresses prolactin; always with anticoagulation prophylaxis) is considered in severe disease (ejection fraction under 25%) and improves recovery in small trials.
• Cardiogenic shock: inotropes, mechanical support, early transplantation referral.
• ICD only if ejection fraction remains 35% or less after 6 months of therapy; wearable defibrillator for the interim.
• Continue therapy for at least 12 to 24 months after recovery, then taper carefully.
• Future pregnancy: contraindicated if ejection fraction remains below 50%; recurrence 20 to 50% even with recovery. Provide contraception counselling (avoid oestrogen).