Aortic valve replacement in a 41-year-old woman with uncorrected tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries: a case report.

Pub Date : 2023-12-06 DOI:10.1186/s40981-023-00674-0

Kazutomo Saito, Yudai Iwasaki, Takahiro Tasaki, Hidehisa Saito, Hiroaki Toyama, Yutaka Ejima, Masanori Yamauchi

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Abstract

Background: Tetralogy of Fallot (TOF) is a complex cyanotic congenital heart disease. As most patients with TOF undergo palliative or radical surgical repair during childhood, cardiac surgery under cardiopulmonary bypass (CPB) for adult survivors with unrepaired TOF is exceedingly rare.

Case presentation: A 41-year-old woman with unrepaired TOF, pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) developed acute infectious endocarditis (IE). As vegetation gradually increased despite intravenous antibiotic administration, she was scheduled for urgent aortic valve replacement under CPB. Pulmonary blood flow was primarily provided by the MAPCAs originating from the descending aorta. Intra-aortic balloon occlusion for MAPCAs was performed to ensure a bloodless surgical field. Aortic valve replacement was successful.

Conclusion: An adult with uncorrected TOF developed acute IE and subsequently had successful cardiac surgery under CPB. Understanding TOF physiology with PA and MAPCAs, particularly pulmonary blood flow through MAPCAs, is crucial.

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一名患有法洛氏四联症、肺动脉闭锁和大动脉-肺动脉侧支的 41 岁女性的主动脉瓣置换术：病例报告。

背景：法洛氏四联症（TOF）是一种复杂的紫绀型先天性心脏病。由于大多数 TOF 患者都是在儿童时期接受姑息性或根治性手术修复，因此在心肺旁路（CPB）下为未修复 TOF 的成年幸存者进行心脏手术极为罕见：一名 41 岁的女性因 TOF 未修复、肺动脉闭锁（PA）和大动脉-肺动脉侧支（MAPCA）而患上急性感染性心内膜炎（IE）。尽管静脉注射了抗生素，但植被仍在逐渐增加，因此她被紧急安排在 CPB 下进行主动脉瓣置换术。肺血流主要由源自降主动脉的 MAPCA 提供。为确保手术区域无血，对 MAPCAs 实施了主动脉内球囊封堵。主动脉瓣置换术非常成功：一名未矫正 TOF 的成人发生了急性 IE，随后在 CPB 下成功进行了心脏手术。了解伴有 PA 和 MAPCA 的 TOF 生理学，尤其是通过 MAPCA 的肺血流至关重要。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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