Maluf M A, Smith M, Abellan D M, Troster E J, Takaoka F, Rati M, Faiwichow G
Department of Social Pediatrics, Hospital Israelita Albert Einstein, São Paulo, Brazil.
Tex Heart Inst J. 1997;24(3):226-9.
Origin of the right coronary artery from the pulmonary artery is a rare lesion occasionally found at angiography or autopsy. We report the rare preoperative diagnosis, in a child, of anomalous origin of the right coronary artery from the pulmonary artery, in association with a ventricular septal defect. The chest radiograph was normal, but auscultation revealed a continuous murmur at the left sternal border and electrocardiography showed right and left ventricular hypertrophy. A transthoracic echocardiogram depicted anomalous origin of the right coronary artery from the pulmonary artery. Color-flow Doppler echocardiography indicated possible right-coronary-artery-to-right-ventricle fistulae. Diagnosis was made by selective left coronary arteriography, which showed retrograde filling of the right coronary artery from collateral vessels. Selective left coronary arteriography depicted intercoronary flow, with no fistulae. Operative repair consisted of moving the proximal right coronary artery from its origin at the pulmonary trunk to the aorta. An associated procedure for correction of the ventricular septal defect was performed. The postoperative cardiac angiogram showed that the ventricular septal defect was closed and that flow through the right coronary artery was normal. Preoperative diagnosis of anomalous origin of the right coronary artery from the pulmonary artery is important, because this condition is surgically correctable.
右冠状动脉起源于肺动脉是一种罕见的病变，偶尔在血管造影或尸检时发现。我们报告了一例儿童罕见的术前诊断，即右冠状动脉起源于肺动脉并伴有室间隔缺损。胸部X线片正常，但听诊发现左胸骨缘有连续性杂音，心电图显示右心室和左心室肥大。经胸超声心动图显示右冠状动脉起源于肺动脉异常。彩色多普勒超声心动图提示可能存在右冠状动脉至右心室瘘。通过选择性左冠状动脉造影进行诊断，显示右冠状动脉由侧支血管逆行充盈。选择性左冠状动脉造影显示冠状动脉间有血流，无瘘管。手术修复包括将右冠状动脉近端从其在肺动脉干的起源处移至主动脉。同时进行了矫正室间隔缺损的相关手术。术后心脏血管造影显示室间隔缺损已闭合，右冠状动脉血流正常。术前诊断右冠状动脉起源于肺动脉异常很重要，因为这种情况可通过手术矫正。
