Catastrophic presentation of peripartum cardiomyopathy: a case report of a challenging diagnosis

Peripartum cardiomyopathy (PPCM) usually affects women in the last month of pregnancy or in the first months following delivery and typically presents with signs and symptoms of heart failure (HF), although catastrophic presentations may be the initial manifestation.

A 36-year-old woman reported intense fatigue that began after delivery. Three months following delivery, she suffered a cardiac arrest at home, in shockable rhythm, with a total estimated time of 70 min before return of spontaneous circulation. Electrocardiogram presented diffuse ST-segment depression and transthoracic echocardiography showed severe left-ventricle (LV) dysfunction with global hypokinesis. In the catheterization laboratory, she underwent a triple rule out (no aortic, coronary, or pulmonary vascular disease); ventriculography was not suggestive of Takotsubo syndrome. She was transferred to the cardiac intensive care unit, under invasive mechanical ventilation and vasopressor support. A presumptive diagnosis of PPCM was made and bromocriptine and heparin were started. In three days, she was weaned from haemodynamic support and extubated, with good neurologic outcome. Cardiac magnetic resonance showed no signs of inflammation or fibrosis. Cardiomyopathy genetic test was negative. PPCM diagnosis was assumed, HF therapy was introduced and a cardioverter-defibrillator was implanted. At 2.5 months follow up, she presented HF NYHA Class II and recovered LV function.

We present a case of a woman, three months after delivery, who developed a catastrophic manifestation of PPCM. This case raises awareness about atypical presentations of PPCM, whose diagnosis should be considered in the appropriate clinical context, but ultimately, remains a diagnosis of exclusion.