Cardiology > Peripartum cardiomyopathy (PPCM)  

Background 

Peripartum cardiomyopathy (PPCM) is an idiopathic form of systolic heart failure that occurs during the last month of pregnancy or within five months postpartum, in women without known structural heart disease prior to the last trimester. It is characterized by left ventricular (LV) systolic dysfunction (EF <45%) and dilated cardiac chambers, leading to signs and symptoms of heart failure. 

II) Classification/Types

By Onset: 

• • Early-onset PPCM: Symptoms begin in the last month of pregnancy. 

• • Late-onset PPCM: Symptoms begin postpartum (up to 5 months). 

By Recovery: 

• • Recovered PPCM: Normalization of LV ejection fraction (EF ≥50%). 

• • Persistent Dysfunction: Ongoing LV systolic dysfunction. 

By Severity (Echocardiographic Parameters): 

• • Mild: EF 40–45% 

• • Moderate: EF 30–39% 

• • Severe: EF <30% 

III) Pathophysiology 

The exact mechanism is unclear, but PPCM likely involves a combination of inflammatory, hormonal, and genetic factors. A proposed mechanism includes oxidative stress leading to cleavage of prolactin into a 16-kDa anti-angiogenic fragment that impairs endothelial function and myocardial capillary perfusion. Myocardial inflammation and autoimmune responses also contribute to ventricular dysfunction. 

IV) Epidemiology

• • Sex: Exclusively affects women. 

• • Age: Most common in women >30 years of age. 

• • Geography: Higher incidence in Africa, Haiti, and parts of Asia. 

• • Comorbidities: Hypertension, preeclampsia, multiparity, malnutrition, and African ancestry increase risk. 

Etiology

I) Causes

PPCM is idiopathic but may be triggered or exacerbated by: 

• • Hemodynamic stress of pregnancy 

• • Oxidative stress and inflammation 

• • Anti-angiogenic hormonal factors (e.g., cleaved prolactin) 

• • Genetic predisposition 

• • Nutritional deficiencies (selenium, iron) 

II) Risk Factors

• • Advanced maternal age (>30 years) 

• • Multiparity 

• • African descent 

• • Preeclampsia or gestational hypertension 

• • Twin/multiple gestation 

• • Prolonged tocolytic use 

• • Use of prolactin-enhancing drugs 

• • Smoking or substance abuse 

 
Clinical Presentation

I) History (Symptoms)

• • Progressive dyspnea, orthopnea, paroxysmal nocturnal dyspnea 

• • Fatigue and exercise intolerance 

• • Lower extremity edema 

• • Palpitations 

• • Cough, especially at night 

• • Syncope or presyncope (if arrhythmia or low cardiac output) 

II) Physical Exam (Signs)

Vital Signs: 

• • Tachycardia 

• • Hypotension (if cardiogenic shock) 

Cardiac Exam: 

• • Displaced apical impulse 

• • S3 gallop 

• • Murmur of functional mitral regurgitation 

Pulmonary: 

• • Bibasilar crackles or rales 

• • Tachypnea 

• • Signs of pulmonary edema 

Peripheral: 

• • Jugular venous distension 

• • Peripheral edema 

• • Hepatomegaly (in severe cases) 

Differential Diagnosis (DDx)

• • Dilated cardiomyopathy 

• • Preexisting heart failure (unrecognized before pregnancy) 

• • Preeclampsia with pulmonary edema 

• • Amniotic fluid embolism 

• • Pulmonary embolism 

• • Sepsis 

• • Anemia-related high-output heart failure 

• • Myocarditis 

Diagnostic Tests

Initial Tests: 

Transthoracic Echocardiogram (TTE): 

• • LV dilation and reduced EF (<45%) 

• • Global hypokinesis 

• • Possible mitral regurgitation 

• • RV dysfunction in severe cases 

Electrocardiogram (ECG): 

• • Sinus tachycardia 

• • Nonspecific ST-T changes 

• • Left atrial enlargement or LVH 

Chest X-ray: 

• • Cardiomegaly 

• • Pulmonary venous congestion or edema 

BNP/NT-proBNP: 

• • Elevated, supporting heart failure diagnosis 

Cardiac MRI: 

• • Can assess myocardial inflammation or fibrosis 

• • Rule out myocarditis 

Laboratory Tests: 

• • CBC, renal and liver function, TSH 

• • Cardiac enzymes (to exclude MI) 

• • Prolactin levels (research setting) 

Treatment

I) Medical Management

Heart Failure Therapy (Pregnancy Considerations): 

• • Diuretics (e.g., furosemide) – for volume overload 

• • Hydralazine + Nitrates – preferred afterload reducers during pregnancy 

• • Beta-blockers (e.g., metoprolol) – safe in pregnancy/postpartum 

• • ACE inhibitors/ARBs – contraindicated in pregnancy, started postpartum if EF remains reduced 

• • Spironolactone – contraindicated in pregnancy; used postpartum if indicated 

Anticoagulation: 

• • Indicated if EF <30% due to thromboembolism risk 

• • LMWH during pregnancy; warfarin postpartum if breastfeeding 

Prolactin Inhibition (Experimental): 

• • Bromocriptine (dopamine agonist): Used in select patients to suppress prolactin; may improve EF when combined with standard therapy 

II) Interventional/Surgical

• • Implantable cardioverter-defibrillator (ICD): Consider in persistent EF <35% after 6 months 

• • Mechanical circulatory support: For refractory cardiogenic shock (e.g., intra-aortic balloon pump, LVAD) 

• • Heart transplantation: Rare, for end-stage unresponsive PPCM 

Patient Education, Screening, Vaccines

Education: 

• • Emphasize importance of medication adherence 

• • Educate on fluid restriction and daily weights 

• • Teach early signs of decompensation (e.g., increased dyspnea, weight gain) 

• • Avoid future pregnancies until EF recovers; high recurrence risk if EF remains reduced 

Lifestyle: 

• • Salt restriction in fluid-overloaded states 

• • Avoid alcohol and smoking 

Vaccinations: 

• • Influenza 

• • COVID-19 

• • Pneumococcal (if immunocompromised) 

Consults

• • Cardiology: For initial diagnosis and long-term management 

• • Maternal-Fetal Medicine (MFM): For co-management during pregnancy 

• • Critical Care: If patient presents with cardiogenic shock 

• • Electrophysiology: For ventricular arrhythmias or consideration of ICD 

• • Lactation Consultant: If bromocriptine therapy or other contraindications to breastfeeding 

Follow-Up

• • Serial Echocardiograms: 

• • • At diagnosis 

• • • Every 3–6 months until EF recovery 

• • • Annually if LV function does not normalize 

• • • Monitor EF and signs of remodeling 

• • Long-Term Care: 

• • • Some patients require lifelong heart failure medications 

• • • Counseling on future pregnancy risks—avoid if EF remains <50%