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左冠状动脉起源于肺动脉的诊断与预后

Diagnosis and prognosis of anomalous origin of the left coronary artery from the pulmonary artery.

作者信息

Holst Line Marie Broksø, Helvind Morten, Andersen Henrik Ørbæk

机构信息

Børneafdelingen, Hvidovre Hospital, Kettegård Alle 30, 2650 Hvidovre, Denmark.

出版信息

Dan Med J. 2015 Sep;62(9).

Abstract

INTRODUCTION

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is an uncommon congenital heart abnormality. The aim of this study was to describe a single-centre experience with surgical repair of this condition.

METHODS

We performed a retrospective analysis of cases from February 2004 to January 2014.

RESULTS

Ten patients presented with the diagnosis of ALCAPA. A total of seven infants and three adults underwent surgical repair in our Department of Thoracic Surgery, Rigshospitalet, Denmark. The seven infants presented with symptoms of heart failure: dyspnoea, sweating or failure to thrive; two adults were asymptomatic and one adult presented with cardiac arrest. Six infants had moderate to severe mitral valve regurgitation and five of these patients had preoperative moderate to severely reduced left ventricular function. Nine patients underwent surgical repair by re-implantation of the left coronary artery to the aorta and one underwent surgical repair ad modus Takeuchi (an aortopulmonary window). None of the patients underwent re-operation and none died.

CONCLUSION

All ten patients survived with recovery of left ventricular function within 12 months. An early diagnosis and prompt surgical intervention is warranted in the treatment of ALCAPA.

摘要

引言

左冠状动脉起源于肺动脉(ALCAPA)是一种罕见的先天性心脏异常。本研究的目的是描述单中心对该疾病进行手术修复的经验。

方法

我们对2004年2月至2014年1月的病例进行了回顾性分析。

结果

10例患者被诊断为ALCAPA。丹麦里格霍斯医院胸外科共有7名婴儿和3名成人接受了手术修复。7名婴儿出现心力衰竭症状:呼吸困难、出汗或发育不良;2名成人无症状,1名成人出现心脏骤停。6名婴儿有中度至重度二尖瓣反流,其中5名患者术前左心室功能中度至重度降低。9例患者通过将左冠状动脉重新植入主动脉进行手术修复,1例患者接受了竹内式手术修复(一种主肺动脉窗手术)。所有患者均未再次手术,也无死亡病例。

结论

所有10例患者均存活,左心室功能在12个月内恢复。对于ALCAPA的治疗,早期诊断和及时的手术干预是必要的。

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