Postpartum Cardiomyopathy Definition and Diagnosis

Core Diagnostic Criteria

Postpartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy presenting with heart failure secondary to left ventricular systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause of heart failure is found, as defined by the European Society of Cardiology 1
The left ventricle may not be dilated, but the ejection fraction is nearly always reduced below 45% 1
Traditionally defined as occurring during the last month of pregnancy or within 5 months postpartum, though this timeframe is considered arbitrary and may lead to under-diagnosis, according to the European Journal of Heart Failure 1, 2
Distinct from other types of heart failure, with a unique clinical course that can include rapid progression to end-stage heart failure or spontaneous recovery, as noted by the European Society of Cardiology 1

Incidence varies worldwide: approximately 1 in 2500-4000 in the USA, 1 in 1000 in South Africa, and 1 in 300 in Haiti, as reported by the European Journal of Heart Failure 1

Most patients (78%) develop symptoms in the first 4 months after delivery, with only 9% presenting in the last month of pregnancy, according to the European Journal of Heart Failure 3
Common symptoms include dyspnea, fatigue, and peripheral edema, which may be mistaken for normal pregnancy symptoms, as noted by the European Society of Cardiology 3
Physical examination may reveal a displaced apical impulse (72%), third heart sound (92%), and mitral regurgitation (43%), as reported by the European Journal of Heart Failure 3
Presentation severity ranges from NYHA class I to IV symptoms, with most patients presenting with class III or IV, according to the European Journal of Heart Failure 3

Oxidative stress activating cathepsin D in cardiomyocytes, which cleaves prolactin into a cardiotoxic 16 kDa fragment, as proposed by the European Journal of Heart Failure 1
This prolactin fragment inhibits endothelial cell proliferation, induces endothelial cell apoptosis, disrupts capillary structures, and impairs cardiomyocyte function, as noted by the European Society of Cardiology 1
Autoimmune responses may play a role, with high titers of auto-antibodies against cardiac tissue proteins found in many PPCM patients, as reported by the European Journal of Heart Failure 2
Potential genetic susceptibility, with some cases possibly representing an initial manifestation of familial dilated cardiomyopathy, according to the European Society of Cardiology 2

Echocardiography is essential, showing reduced left ventricular ejection fraction (<45%), as recommended by the European Society of Cardiology 1
Electrocardiogram often shows abnormalities (ST-T wave changes in 96%, LV hypertrophy voltage criteria in 66%), as reported by the European Journal of Heart Failure 3
B-type natriuretic peptide (BNP) or NT-proBNP levels are typically elevated, according to the European Society of Cardiology 3
Cardiac MRI provides more accurate assessment of chamber volumes and ventricular function, and can detect LV thrombus, as noted by the European Journal of Heart Failure 3
Differential diagnosis must exclude pre-existing cardiac conditions, hypertensive heart disease, pulmonary embolism, and other causes of heart failure, as recommended by the European Society of Cardiology 4

Left ventricular thrombosis is common in patients with LVEF <35%, leading to risk of systemic emboli, as reported by the European Journal of Heart Failure 3