Najm H K, Williams W G, Coles J G, Rebeyka I M, Freedom R M
Department of Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
Ann Thorac Surg. 1997 Mar;63(3):669-75. doi: 10.1016/s0003-4975(96)01366-5.
Children with pulmonary atresia and an intact ventricular septum show a heterogeneous spectrum of cardiac anomalies. A biventricular repair is attainable in some; a Fontan procedure or a one-and-a-half ventricle is the only possible repair for others. Children with right ventricle-to-coronary artery connections, with or without right ventricle-dependent coronary artery blood flow, are a high-risk group.
Between May 1980 and December 1994, 22 children underwent a Fontan operation for the treatment of pulmonary atresia with an intact ventricular septum at The Hospital for Sick Children, Toronto. The mean age was 5.8 years (median, 4.9 years). All children had had at least one pre-Fontan palliative procedure; 19 had two, and 7 of these had three or more. Right ventricle-to-coronary artery connections were present in 15 children, including 5 with right ventricle-dependent coronary artery blood flow. Thromboexclusion of the right ventricle was done in 10 children, with 7 undergoing it before and 3 at the time of the Fontan procedure.
There were three early deaths (13.6%) and one late death. The actuarial survival at 10 years after the Fontan operation was 80%. Early postoperative complications occurred in 4 children. Follow-up was completed in all children at a mean of 4 years (range, 1 to 12.5 years) after the Fontan operation. Atrial arrhythmia occurred in 3 children, and permanent pacemakers were required in 4.
Results of the Fontan operation for the treatment of pulmonary atresia with an intact ventricular septum are satisfactory. Thromboexclusion of the right ventricle is indicated in the presence of right ventricle-to-coronary artery connections without right ventricle-dependent coronary artery blood flow. The right ventricle should not be decompressed or thromboexcluded in children with right ventricle-dependent coronary artery blood flow, and at the Fontan operation, saturated blood must enter the right ventricle.
患有肺动脉闭锁且室间隔完整的儿童表现出一系列不同的心脏异常。部分患儿可实现双心室修复；而对于其他患儿，Fontan手术或一期半心室修复是唯一可行的修复方式。存在右心室至冠状动脉连接且伴有或不伴有右心室依赖型冠状动脉血流的患儿属于高危组。
1980年5月至1994年12月期间，22例患有肺动脉闭锁且室间隔完整的患儿在多伦多病童医院接受了Fontan手术治疗。平均年龄为5.8岁（中位数为4.9岁）。所有患儿在Fontan手术前至少接受过一次姑息性手术；19例接受过两次，其中7例接受过三次或更多次。15例患儿存在右心室至冠状动脉连接，其中5例伴有右心室依赖型冠状动脉血流。10例患儿进行了右心室血栓清除术，7例在Fontan手术前进行，3例在Fontan手术时进行。
有3例早期死亡（13.6%）和1例晚期死亡。Fontan手术后10年的精算生存率为80%。4例患儿出现早期术后并发症。所有患儿在Fontan手术后平均4年（范围为1至12.5年）完成随访。3例患儿发生房性心律失常，4例需要植入永久性起搏器。
Fontan手术治疗肺动脉闭锁且室间隔完整的效果令人满意。对于存在右心室至冠状动脉连接但无右心室依赖型冠状动脉血流的患儿，建议进行右心室血栓清除术。对于伴有右心室依赖型冠状动脉血流的患儿，不应使右心室减压或进行血栓清除，且在Fontan手术时，饱和血液必须进入右心室。
