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围产期心肌病作为未确诊镰状细胞病的首发表现:一例报告

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

作者信息

Pallangyo Pedro, Mkojera Zabella, Mfanga Loveness, Komba Makrina, Kisenge Peter R

机构信息

Jakaya Kikwete Cardiac Institute, Dar es Salaam, Tanzania.

出版信息

J Investig Med High Impact Case Rep. 2025 Jan-Dec;13:23247096251346829. doi: 10.1177/23247096251346829. Epub 2025 May 31.

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.

摘要

发生在镰状细胞病(SCD)背景下的围产期心肌病(PPCM)极为罕见,尤其是作为未确诊SCD的初始表现。我们报告了一例39岁的非洲初产妇,妊娠36周,出现了1周的心力衰竭症状。她的产前过程除了严重贫血外基本顺利,为此她输注了2单位全血,随后开具了补血药。实验室检查显示溶血性贫血、镰状试验阳性,血红蛋白电泳证实镰状血红蛋白占93%,胎儿血红蛋白占4.1%,确诊为纯合子SCD。经胸超声心动图显示左心室射血分数严重降低(LVEF 26%),左心腔扩大,符合PPCM的诊断。她被收入产科重症监护病房,由多学科团队进行管理,通过支持治疗病情稳定。38周时,她成功进行了择期剖宫产,产后恢复顺利。产后9天,她病情稳定(纽约心脏协会II级)出院,接受指南指导的药物治疗,并参加门诊随访。6个月时,随访超声心动图显示心脏功能部分恢复,LVEF为38%。她继续由心脏病学和血液学团队进行监测。该病例强调了在出现不明原因心力衰竭的孕妇中考虑潜在血红蛋白病的至关重要性,特别是在SCD流行的地区。

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本文引用的文献

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Sickle Cell Disease in Sub-Saharan Africa.撒哈拉以南非洲地区的镰状细胞病
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