Di Donato R M, Wernovsky G, Walsh E P, Colan S D, Lang P, Wessel D L, Jonas R A, Mayer J E, Castañeda A R
Department of Cardiology, Children's Hospital, Boston, MA 02115.
Circulation. 1989 Dec;80(6):1689-705. doi: 10.1161/01.cir.80.6.1689.
Between January 1983 and December 1987, 62 patients underwent an arterial switch operation for transposition of the great arteries with ventricular septal defect or double outlet right or left ventricle. There were three hospital deaths (4.8%), and no deaths occurred in neonates (less than 1 month of age, n = 18). There were three late deaths, one due to coronary obstruction and two due to pulmonary vascular obstructive disease. One child has been lost to follow-up. We have prospectively evaluated the remaining 55 survivors by clinical evaluation, echocardiography, cardiac catheterization, ambulatory electrocardiographic monitoring, and limited electrophysiologic studies. The mean length of follow-up has been 27 +/- 16 months since surgery. One child has required reoperation for a residual ventricular septal defect; no child has undergone reoperation for supravalvar pulmonary or aortic stenosis. Aortic regurgitation was identified in 12 children (22%), which was mild in 11 and moderate in one. One child has asymptomatic occlusion of the left main coronary artery, one child has a tiny right coronary artery-to-pulmonary artery fistula, and one child has abnormal left ventricular wall motion according to follow-up angiography. No other abnormalities of systemic (left) ventricular function have been identified at late follow-up. In addition to the two late deaths due to pulmonary vascular obstructive disease, three children, all of whom were repaired at more than 6 months of age, have elevated pulmonary vascular resistance. Notable postoperative arrhythmias include complete heart block in four patients and nonsustained supraventricular or ventricular tachycardia early after surgery in eight patients (all resolved without medication at later follow-up). Only two patients have evidence of sinus node dysfunction and have not required treatment. The low hospital mortality and encouraging early follow-up data represent a significant improvement over atrial level repairs, supporting the arterial switch operation as the procedure of choice for children who have transposition of the great arteries with ventricular septal defect or double outlet ventricle. Because of the potential for the development of early pulmonary vascular obstructive disease in these patients, repair is recommended within the first 2 months of life.
1983年1月至1987年12月期间，62例患有大动脉转位合并室间隔缺损或右或左心室双出口的患者接受了动脉调转手术。有3例医院死亡（4.8%），新生儿（小于1个月龄，n = 18）无死亡。有3例晚期死亡，1例死于冠状动脉阻塞，2例死于肺血管阻塞性疾病。有1名儿童失访。我们通过临床评估、超声心动图、心导管检查、动态心电图监测和有限的电生理研究对其余55名幸存者进行了前瞻性评估。自手术以来，平均随访时间为27±16个月。1名儿童因残余室间隔缺损需要再次手术；没有儿童因肺动脉瓣上或主动脉瓣上狭窄接受再次手术。12名儿童（22%）发现有主动脉瓣反流，其中11例为轻度，1例为中度。1名儿童左主冠状动脉无症状性闭塞，1名儿童有微小的右冠状动脉至肺动脉瘘，1名儿童根据随访血管造影显示左心室壁运动异常。晚期随访未发现其他体循环（左）心室功能异常。除了2例因肺血管阻塞性疾病导致的晚期死亡外，3例均在6个月以上接受修复的儿童肺血管阻力升高。值得注意的术后心律失常包括4例患者出现完全性心脏传导阻滞，8例患者在术后早期出现非持续性室上性或室性心动过速（所有患者在后期随访中无需药物治疗即可缓解）。只有2例患者有窦房结功能障碍的证据且无需治疗。低医院死亡率和令人鼓舞的早期随访数据表明，与心房水平修复相比有显著改善，支持动脉调转手术作为患有大动脉转位合并室间隔缺损或双出口心室儿童的首选手术方法。由于这些患者有早期发生肺血管阻塞性疾病的可能，建议在出生后2个月内进行修复。
