Fyfe D A, Edwards W D, Driscoll D J
J Am Coll Cardiol. 1986 Aug;8(2):402-6. doi: 10.1016/s0735-1097(86)80058-4.
Children who die after operation for pulmonary atresia and intact ventricular septum may have myocardial ischemia. The relation between histologic evidence of myocardial ischemic injury and the presence of a right ventricle to coronary artery fistula, coronary artery dysplasia and operation in 17 autopsy specimens was assessed. Age at death ranged from 1 day to 16 years (median, 11 days). Of the 17 hearts, 6 (35%) had right ventricle to coronary artery fistulas, 5 of which had coronary artery dysplasia. In three cases, there was segmental or complete absence of a coronary artery. Ischemia was present in four of these six hearts, two of which had right ventricular outflow reconstruction. Six of the 11 hearts without right ventricle to coronary artery fistulas also had myocardial ischemia. Of these six cases, four had right ventricular outflow reconstruction and two had shunt operations. Death occurred from 1 to 8 days (mean 3) after operation. Hearts with pulmonary atresia and intact ventricular septum may have myocardial ischemia with or without either right ventricle to coronary artery fistulas or coronary artery dysplasia. Myocardial ischemia may occur after right ventricular outflow reconstruction or shunt operations. Thus, myocardial ischemia occurs commonly in patients with pulmonary atresia and intact ventricular septum and is not always related to coronary abnormalities or operation.
患有肺动脉闭锁且室间隔完整的患儿术后死亡可能存在心肌缺血。对17例尸检标本中，心肌缺血性损伤的组织学证据与右心室至冠状动脉瘘、冠状动脉发育异常及手术之间的关系进行了评估。死亡年龄从1天至16岁不等（中位数为11天）。在这17颗心脏中，6例（35%）存在右心室至冠状动脉瘘，其中5例伴有冠状动脉发育异常。3例存在节段性或完全性冠状动脉缺如。这6颗心脏中有4颗存在缺血，其中2颗进行了右心室流出道重建。11颗没有右心室至冠状动脉瘘的心脏中有6颗也存在心肌缺血。在这6例中，4例进行了右心室流出道重建，2例进行了分流手术。术后1至8天（平均3天）死亡。患有肺动脉闭锁且室间隔完整的心脏可能存在心肌缺血，无论有无右心室至冠状动脉瘘或冠状动脉发育异常。右心室流出道重建或分流手术后可能发生心肌缺血。因此，心肌缺血在患有肺动脉闭锁且室间隔完整的患者中很常见，并不总是与冠状动脉异常或手术相关。
