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一名76岁女性患“晚发型成人型”左冠状动脉起源于肺动脉综合征的病例。

A case of "late adult type" of ALCAPA syndrome in a 76-year-old woman.

作者信息

De Stefano Domenico, Bitonti Maria Teresa, Vertulli Daniele, Zobel Bruno Beomonte

机构信息

Unit of Diagnostic Imaging and Interventional Radiology, Departmental Faculty of Medicine and Surgery, Università Campus Bio-Medico di Roma, Via Alvaro del Portillo, 21, 00128 Rome, Italy.

出版信息

J Cardiol Cases. 2024 Jun 15;30(3):79-82. doi: 10.1016/j.jccase.2024.05.006. eCollection 2024 Sep.

Abstract

UNLABELLED

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare condition; late adult presentation is extremely rare but, with modern diagnostics, more asymptomatic or mildly symptomatic elderly patients with ALCAPA are found. A 76-year-old woman was admitted to our emergency department in December 2021 with fever, fatigue, cognitive-motor slowing, and hyposthenia of left hemisoma. Cranium computed tomography (CT) was performed, with no signs of stroke. Contrast-enhanced CT, performed with electrocardiogram-gated technique, showed a dilatated left coronary artery arising directly from the main pulmonary artery; right coronary artery was markedly dilated and tortuous and characterized by multiple inter-coronary collateral arteries with left coronary artery, in particular in the retro-aortic side and along the cone artery. ALCAPA syndrome represents one of the most common causes of myocardial ischemia in children and, if left untreated, results in a mortality rate of up to 80-90 % within the first year of life. In our case, no previous history of cardiological disease was found that could have suggested this type of condition. This is extremely rare, especially if we consider that our patient reached the 8th decade of life.

LEARNING OBJECTIVES

•ALCAPA syndrome can present in adulthood.•Collaterals between coronary arteries are essential to guarantee the survival into adulthood.•Cardiac computed tomography imaging is essential for the diagnosis.

摘要

未标注

左冠状动脉起源于肺动脉(ALCAPA)是一种罕见疾病;成年晚期出现这种情况极为罕见,但随着现代诊断技术的发展,发现了更多无症状或症状轻微的老年ALCAPA患者。一名76岁女性于2021年12月因发热、疲劳、认知运动迟缓及左侧半身肌无力入住我院急诊科。进行了头颅计算机断层扫描(CT),未发现中风迹象。采用心电图门控技术进行的增强CT显示,左冠状动脉直接起源于主肺动脉;右冠状动脉明显扩张且迂曲,其特征是与左冠状动脉之间存在多条冠状动脉侧支,特别是在主动脉后侧和沿圆锥动脉处。ALCAPA综合征是儿童心肌缺血最常见的原因之一,如果不治疗,在出生后第一年内死亡率高达80 - 90%。在我们的病例中,未发现先前有任何可能提示这种疾病的心脏病史。这极为罕见,尤其是考虑到我们的患者已步入八十岁。

学习目标

•ALCAPA综合征可在成年期出现。•冠状动脉之间的侧支对于保证成年后的生存至关重要。•心脏计算机断层扫描成像对于诊断至关重要。

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