Ramoğlu Mehmet Gökhan, Bulut Mustafa Orhan, Epçaçan Serdar, Dedemoğlu Mehmet
Department of Pediatric Cardiology, University of Health Sciences, Van Training and Research Hospital, Van, Turkey.
Department of Pediatric Cardiology, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Turkey.
Turk Gogus Kalp Damar Cerrahisi Derg. 2019 Jun 14;27(3):388-391. doi: 10.5606/tgkdc.dergisi.2019.16320. eCollection 2019 Jul.
Anomalous origin of left coronary artery from pulmonary artery syndrome is a rare, but severe congenital cardiac malformation. It is an important cause of dilated cardiomyopathy and left heart failure during infancy and, if left untreated, the prognosis is poor with an overall mortality rate over 90%. About 15% of patients can survive beyond the first year of life, depending on the development of collateral circulation and may present with angina, dyspnea, syncope, and arrhythmias. Myocardial infarction and sudden cardiac death may be the only and the first symptom in some cases. The treatment of choice for this syndrome is urgent surgical intervention with favorable long-term outcomes. Herein, we present an asymptomatic adolescent active sportsman who was diagnosed with anomalous origin of left coronary artery from pulmonary artery syndrome and underwent a successful surgery.
左冠状动脉起源于肺动脉综合征是一种罕见但严重的先天性心脏畸形。它是婴儿期扩张型心肌病和左心衰竭的重要原因，如果不治疗，预后很差，总死亡率超过90%。约15%的患者可存活至一岁以后，这取决于侧支循环的发育情况，可能会出现心绞痛、呼吸困难、晕厥和心律失常。在某些情况下，心肌梗死和心源性猝死可能是唯一且首发的症状。该综合征的首选治疗方法是紧急手术干预，长期效果良好。在此，我们报告一例无症状的青少年活跃运动员，他被诊断为左冠状动脉起源于肺动脉综合征，并接受了成功的手术。
