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先天性主肺动脉窗合并动脉导管未闭妊娠的致命结局

Fatal outcome of congenital aortopulmonary window with patent ductus arteriosus complicating pregnancy.

作者信息

Thirunavukkarasu Balamurugan, Kithan Lijanthung S, Kumar Nikhil, Jain Arihant, Bal Amanjit

机构信息

Post Graduate Institute of Medical Education & Research (PGIMER), Departments of Histopathology, Chandigarh, India.

Post Graduate Institute of Medical Education & Research (PGIMER), Departments of Internal Medicine, Chandigarh, India.

出版信息

Autops Case Rep. 2021 Apr 15;11:e2021265. doi: 10.4322/acr.2021.265.

Abstract

Aortopulmonary window (APW) is a rare congenital heart defect with abnormal communication between the ascending aorta and the pulmonary trunk with two separate semilunar valves. We present an autopsy case report wherein a young primigravida woman presented with progressive breathlessness and central cyanosis at 21 weeks of gestation. Echocardiography performed in the emergency room revealed elevated right-sided cardiac pressures suggestive of severe pulmonary hypertension; however, no structural cardiac defect was discernible. The patient succumbed to congestive cardiac failure and progressive hypoxia within 5 days of hospitalization. The autopsy revealed a Type I aortopulmonary window (2 cm) with patent ductus arteriosus. The lungs showed changes of severe pulmonary hypertension with superadded bronchopneumonia. This report underscores a rare presentation of APW, undiagnosed until pregnancy, leading to the Eisenmenger syndrome and death.

摘要

主肺动脉窗(APW)是一种罕见的先天性心脏缺陷,升主动脉与肺动脉干之间存在异常交通,且有两个独立的半月瓣。我们报告一例尸检病例,一名初产妇在妊娠21周时出现进行性呼吸困难和中央性发绀。急诊室进行的超声心动图显示右侧心腔压力升高,提示严重肺动脉高压;然而,未发现结构性心脏缺陷。患者在住院5天内死于充血性心力衰竭和进行性缺氧。尸检发现一个2厘米的I型主肺动脉窗,伴有动脉导管未闭。肺部显示出严重肺动脉高压合并支气管肺炎的改变。本报告强调了主肺动脉窗一种罕见的表现形式,在妊娠前未被诊断,导致艾森曼格综合征并死亡。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2e3/8087394/407c55e09623/autopsy-11-e2021265-gf01.jpg

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