Peripartum Cardiomyopathy

Peripartum cardiomyopathy (PPCM) is a rare cause of cardiomyopathy that occurs during late pregnancy or the early postpartum period. This condition can be life-threatening and is characterized by significant left ventricular dysfunction and heart failure. However, PPCM has had various definitions. In 2000, The National Heart Lung and Blood Institute and The Office of Rare Diseases workshop established the following 4 criteria for PPCM, becoming the condition's first case definition: Cardiac failure in the last month of pregnancy or within 5 months of delivery. Absence of a determinable etiology for the cardiac failure. Absence of demonstrable structural cardiac disease before the last month of pregnancy. Echocardiographic evidence of diminished left ventricular systolic function noted by one of the following: Left ventricular ejection fraction (LVEF) <45%. Motion-mode fractional shortening <30%. Left ventricular end-diastolic dimension exceeding 2.7 cm/m Then, the 2019 European Society of Cardiology Working Group used the following 3 key criteria: Development of heart failure toward the end of pregnancy or in the postpartum period. Absence of another identifiable cause of heart failure. Left ventricular systolic dysfunction with a left ventricle (LV) ejection fraction <45%; LV may or may not be dilated . An expanded definition of PPCM has been proposed to include 2 additional categories: early PPCM, which is diagnosed from the first to the ninth month of pregnancy, and late PPCM, which is identified between 6 and 12 months after delivery. This broader framework acknowledges variations in the timing of disease onset and extends beyond the traditional postpartum focus. Patients with PPCM typically present with dyspnea, fatigue, cough, orthopnea, paroxysmal nocturnal dyspnea, pedal edema, and hemoptysis, though clinicians may not promptly recognize the condition as these symptoms are nonspecific. The primary diagnostic studies used to assess whether peripartum patients meet the definition of PPCM are the electrocardiogram (ECG) and echocardiogram. Though peripartum cardiomyopathy is acutely treated similarly to other forms of systolic heart failure with pregnancy-safe therapies (eg, dietary sodium restriction, loop diuretics, beta-blockers, hydralazine or nitrates, and digoxin), the overall management of cardiomyopathy associated with pregnancy requires the collaboration of a multidisciplinary team to address the various aspects of patient care the condition affects including labor and delivery, lactation, contraception, and family planning. Though many patients may recover depending on their left ventricular ejection fraction at the time of diagnosis, the condition is associated with many adverse outcomes, including brain injury, cardiopulmonary arrest, pulmonary edema, and death. Therefore, prompt evaluation and treatment are essential to improving patient outcomes.