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主动脉异常起源的左肺动脉:先天性心脏病中的罕见实体。

Anomalous Origin of Left Pulmonary Artery From the Aorta: A Rare Entity in Congenital Heart Disease.

机构信息

Division of Pediatric Cardiology, Washington University in St. Louis School of Medicine and St. Louis Children's Hospital, St. Louis, Missouri.

Division of Pediatric Cardiology, Washington University in St. Louis School of Medicine and St. Louis Children's Hospital, St. Louis, Missouri.

出版信息

Ann Thorac Surg. 2019 Mar;107(3):e181-e182. doi: 10.1016/j.athoracsur.2018.07.083. Epub 2018 Sep 29.

Abstract

Anomalous origin of the left pulmonary artery (ALPA) from the aorta is a rare form of congenital heart disease, leading to long-term morbidity if not treated early. There is a reported association of this anomaly with 22q11 microdeletion syndrome. Surgical repair is commonly performed by direct reimplantation to the main pulmonary artery with or without patch or flap techniques. This report presents two unique cases of ALPA, describing embryologic considerations for morphogenesis of ALPA with 22q11 microdeletion syndrome and a novel surgical technique involving unroofing of the unique anatomy of the common wall between the left and main pulmonary arteries.

摘要

左肺动脉(ALPA)异常起源于主动脉是一种罕见的先天性心脏病,如果不早期治疗,会导致长期发病。据报道,这种异常与 22q11 微缺失综合征有关。手术修复通常通过直接再植入主肺动脉来完成,可采用补片或皮瓣技术。本报告介绍了两个独特的 ALPA 病例,描述了 22q11 微缺失综合征中 ALPA 形态发生的胚胎学考虑因素,以及一种涉及左肺动脉和主肺动脉之间共同壁的独特解剖结构开窗的新手术技术。

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