Tam C K, Lightfoot N E, Finlay C D, Coles J, Williams W G, Trusler G A, Freedom R M
Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
Am J Cardiol. 1989 Mar 1;63(9):589-93. doi: 10.1016/0002-9149(89)90904-1.
Tricuspid atresia is an uncommon form of congenital heart disease and long-term survival was rare before the Fontan era. It was thought that the long-term survival of patients with tricuspid atresia would be improved by the introduction of the Fontan procedure and its subsequent modifications. This study reviews the clinical course of 84 patients with tricuspid atresia identified in the first year of life in the Fontan era. Prior palliative operations, their results and their ultimate application for the Fontan procedure were considered. Eleven patients died before surgical intervention and 5 did not undergo catheterization or echocardiographic confirmation before death. Five children underwent the Fontan procedure without prior palliation and 1 child does not require palliation at the present time. Sixty-seven patients (80%) had surgical procedures before evaluation for the suitability of a Fontan operation. Thirty-four patients had a second surgical palliation and 9 patients had a third palliation. The surgical mortalities for the first, second and third palliative surgery were 17.9, 17.6 and 0%, respectively. Thirty-two patients (38%) underwent the Fontan procedure and 2 deaths occurred (6%). An estimate of the probability of surviving for 1 year was 64% (95% confidence limits 54 to 74%) and that of 8 years was 55% (95% confidence limits 44 to 66%).
三尖瓣闭锁是一种少见的先天性心脏病形式，在Fontan手术时代之前，长期存活极为罕见。人们认为，Fontan手术及其后续改良术式的引入会改善三尖瓣闭锁患者的长期存活率。本研究回顾了Fontan手术时代出生后第一年确诊的84例三尖瓣闭锁患者的临床病程。考虑了既往的姑息性手术、其结果以及它们最终用于Fontan手术的情况。11例患者在手术干预前死亡，5例在死亡前未接受心导管检查或超声心动图确诊。5例儿童未行姑息治疗即接受了Fontan手术，1例儿童目前不需要姑息治疗。67例患者（80%）在评估Fontan手术的适用性之前接受了手术。34例患者接受了第二次姑息性手术，9例患者接受了第三次姑息性手术。第一次、第二次和第三次姑息性手术的手术死亡率分别为17.9%、17.6%和0%。32例患者（38%）接受了Fontan手术，2例死亡（6%）。1年生存率估计为64%（95%置信区间54%至74%），8年生存率估计为55%（95%置信区间44%至66%）。
