Peripartum Cardiomyopathy as the Initial Manifestation of Undiagnosed Sickle Cell Disease: A Case Report.

Abstract

Peripartum cardiomyopathy (PPCM) occurring in the context of sickle cell disease (SCD) is exceedingly rare, particularly as the initial presentation of undiagnosed SCD. We report the case of a 39-year-old African primigravida at 36 weeks of gestation who presented with a 1-week history of heart failure symptoms. Her antenatal course was largely uneventful apart from severe anemia, for which she was transfused 2 units of whole blood followed by prescription of hematinics. Laboratory investigations revealed hemolytic anemia, a positive sickling test, and confirmatory hemoglobin electrophoresis demonstrating 93% sickle hemoglobin and 4.1% hemoglobin fetal, establishing a new diagnosis of homozygous SCD. Transthoracic echocardiography revealed a severely reduced left ventricular ejection fraction (LVEF 26%) and dilated left heart chambers, consistent with a diagnosis of PPCM. She was admitted to the maternity intensive care unit and managed by a multidisciplinary team, with stabilization on supportive therapy. At 38 weeks, she underwent a successful elective cesarean section, followed by an uneventful postpartum recovery. She was discharged 9 days postpartum in a stable condition (New York Heart Association Class II) on guideline-directed medical therapy and enrolled in outpatient follow-up. At 6 months, follow-up echocardiography showed partial recovery of cardiac function with an LVEF of 38%. She continues to be monitored by cardiology and hematology teams. This case highlights the critical importance of considering underlying hemoglobinopathies in pregnant patients presenting with unexplained heart failure, especially in regions where SCD is endemic.