Thirunavukkarasu Balamurugan, Kithan Lijanthung S, Kumar Nikhil, Jain Arihant, Bal Amanjit
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.
Autops Case Rep. 2021 Apr 15;11:e2021265. doi: 10.4322/acr.2021.265.
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.
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。