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冠状动脉先天性异常。

Congenital anomalies of the coronary arteries.

作者信息

Silva Andrea, Baptista Maria João, Araújo Emanuel

机构信息

Faculdade de Medicina da Universidade do Porto, Portugal.

Serviço da Cardiologia Pediátrica, Centro Hospital São João, Porto, Portugal; Departamento de Pediatria, Faculdade de Medicina da Universidade do Porto, Porto, Portugal.

出版信息

Rev Port Cardiol (Engl Ed). 2018 Apr;37(4):341-350. doi: 10.1016/j.repc.2017.09.015. Epub 2018 Feb 26.

Abstract

Congenital coronary artery anomalies are modifications of their origin, course or structure and its incidence varies between 0,2 and 5,6% of the general population. Although the majority is asymptomatic, they are the second leading cause of sudden cardiac death in young athletes. The aim of this study is to highlight the main anomalies with hemodynamic significance, including the anomalous origin of a coronary artery from the opposite sinus and anomalous origin of the left coronary artery from pulmonary artery. The anomalous aortic origin of a coronary artery from the opposite sinus accounts for 14-16% of all cardiac deaths, that unexpectedly occur in healthy children or young athletes during or immediately after exercise. The mechanism responsible for the compression/occlusion of the coronary artery originating from the opposite sinus is still unclear and there are several proposed mechanisms. The clinical presentation of these patients is variable and physical examination is normal in most individuals. Transthoracic echocardiogram is the diagnostic test most commonly used. Treatment and management are controversial topic. The anomalous origin of the left coronary artery from pulmonary artery is a very rare condition and without surgery, most infants die within the first 12 months of life. The echocardiogram is also the method of choice for diagnostic confirmation. The diagnosis of this congenital anomaly in an infant, usually seriously ill, is an indication for urgent surgery. Because these anomalies produce hemodynamic changes, it is important an early diagnosis and treatment.

摘要

先天性冠状动脉异常是指其起源、走行或结构的改变,在普通人群中的发生率在0.2%至5.6%之间。虽然大多数患者无症状,但它们是年轻运动员心源性猝死的第二大主要原因。本研究的目的是突出具有血流动力学意义的主要异常情况,包括冠状动脉起源于对侧窦以及左冠状动脉起源于肺动脉。冠状动脉起源于对侧窦的主动脉异常占所有心源性死亡的14%至16%,这些死亡意外发生在健康儿童或年轻运动员运动期间或运动后不久。导致起源于对侧窦的冠状动脉受压/闭塞的机制仍不清楚,有几种机制被提出。这些患者的临床表现各异,大多数人的体格检查正常。经胸超声心动图是最常用的诊断检查。治疗和管理是有争议的话题。左冠状动脉起源于肺动脉是一种非常罕见的情况,未经手术治疗,大多数婴儿在出生后的头12个月内死亡。超声心动图也是确诊的首选方法。对于通常病情严重的婴儿,诊断出这种先天性异常是紧急手术的指征。由于这些异常会产生血流动力学变化,早期诊断和治疗很重要。

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