成人无症状患者一例罕见ALCAPA伴异常侧支循环一例报告

Zahra Nassereddine
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摘要

左冠状动脉异常起源于肺动脉(ALCAPA)综合征是一种罕见的先天性异常,其中左主冠状动脉起源于肺动脉(PA),而右冠状动脉(RCA)正常起源于主动脉。出生后不久肺血管阻力(PVR)的迅速下降逆转了流向肺动脉的血流,导致左向右分流和严重的左心室(LV)缺血。如果不及时治疗,这种先天性异常会导致过早死亡。极少数情况下,由于侧支循环的发展,一些患者通常从狂喜的RCA存活到成年。大多数成年ALCAPA患者平均死亡年龄为35岁。文献中很少有病例能存活到40多岁或50多岁。我们提出一例无症状的54岁女性ALCAPA,她发现有异常的侧支循环,证明没有明显症状。我们的病人接受了手术矫正,效果很好。我们还回顾了文献中报道的ALCAPA病例,并总结了治疗策略。学习目标:认识到ALCAPA是成人甚至无症状个体的冠状动脉异常之一,并根据现有文献认识到最佳诊断方式和最佳治疗方法。
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A Rare Case of ALCAPA in Adult Asymptomatic Patient and Unusual Collateral Circulation: A Case Report
Anomalous Origin of the Left Coronary Artery from Pulmonary Artery (ALCAPA) syndrome is a rare congenital anomaly where the left main coronary artery arises from the pulmonary artery (PA) and the right coronary artery (RCA) originates normally from the aorta. The rapid decrease in pulmonary vascular resistance (PVR) soon after birth reverses the blood flow to the PA resulting in a left to right shunt and severe left ventricle (LV) ischemia. Left untreated, this congenital anomaly is associated with premature death. Rarely, some patients can survive to adult age due to development of collateral circulation usually from an ecstatic RCA. Most adult patients with ALCAPA die at mean age of 35 years. Very few cases were reported in the literature to survive until late 40s or 50s. We present the case of ALCAPA in an asymptomatic 54-year-old woman who found to have unusual collateral circulation justifying the absence of significant symptoms. Our patient underwent surgical correction with good results. We also review cases of ALCAPA that has been reported in the literature and we summarize treatment strategies. Learning objective: recognition of ALCAPA as one of coronary anomalies in adults even in asymptomatic individuals and recognition of the best diagnostic modality and the optimal management according to available literature.
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