Sano T, Riesenfeld T, Karl T R, Wilkinson J L
Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
Circulation. 1995 Nov 1;92(9 Suppl):II272-8. doi: 10.1161/01.cir.92.9.272.
Limited information is available concerning long-term results, especially systemic right ventricular (RV) or tricuspid valvular function, after intracardiac repair of anomalies associated with discordant atrioventricular (AV) and ventriculoarterial (VA) connections ("congenitally corrected transposition of the great arteries").
We retrospectively reviewed the intermediate-term follow-up of 28 patients, totaling 158 patient-years (median, 60 months), after intracardiac repair involving closure of a ventricular septal defect (VSD) with or without additional surgery. Seven patients had VSD closure alone, 5 had VSD repair with pulmonary stenosis relief, and 16 had VSD closure with conduit insertion between left ventricle and main pulmonary artery. Hospital mortality was 4% (1 of 28 patients; 70% confidence limits, 0.07% to 12%) and the 1-, 5-, and 10-year actuarial survival probabilities were 89%, 83%, and 83%, respectively. Twenty-one of 24 long-term survivors were in New York Heart Association functional class I and 3 were in class II. Sixteen of 24 patients showed increasing tricuspid regurgitation (TR) of more than moderate degree, which occurred within 3 years after surgery in 7 patients. Twelve of 22 patients showed deterioration of RV pump function, mainly (9 of 12 patients) within 3 years postoperatively. The pulmonary to systemic flow ratio at the preoperative cardiac catheter study was significantly (P < .05) higher in patients who developed RV dysfunction (2.3 +/- 1.0, mean +/- SD) than in those with well-maintained RV function (1.4 +/- 0.6).
Intermediate-term results of intracardiac repair for AV and VA discordance were satisfactory in terms of survival and clinical functional status; however, there is concern about systemic RV dysfunction with development of TR relatively early after the operation. Alternative surgical approaches such as anatomic correction or Fontan repair for cases unsuitable for biventricular repair may improve the long-term results, including ventricular and valvular function.
关于“先天性矫正型大动脉转位”（即房室和心室动脉连接不一致的心脏内修复术后的长期结果，尤其是体循环右心室或三尖瓣功能）的信息有限。
我们回顾性分析了28例患者的中期随访情况，总计158患者年（中位数为60个月），这些患者均接受了心脏内修复术，包括有或无附加手术的室间隔缺损（VSD）闭合术。7例患者仅进行了VSD闭合术，5例患者进行了VSD修复并解除了肺动脉狭窄，16例患者进行了VSD闭合术并在左心室和主肺动脉之间插入了导管。住院死亡率为4%（28例患者中的1例；70%置信区间为0.07%至12%），1年、5年和10年的精算生存率分别为89%、83%和83%。24例长期存活者中有21例纽约心脏协会心功能分级为I级，3例为II级。24例患者中有16例出现了中重度以上的三尖瓣反流（TR）加重，其中7例在术后3年内发生。22例患者中有12例出现右心室泵功能恶化，主要（12例患者中的9例）发生在术后3年内。术前心导管检查时，发生右心室功能障碍的患者的肺循环与体循环血流量比值（2.3±1.0，均值±标准差）显著（P<0.05）高于右心室功能维持良好的患者（1.4±0.6）。
就生存率和临床功能状态而言，房室和心室动脉不一致的心脏内修复术的中期结果令人满意；然而，术后相对早期出现的TR发展导致的体循环右心室功能障碍令人担忧。对于不适合双心室修复的病例，解剖矫正或Fontan修复等替代手术方法可能会改善长期结果，包括心室和瓣膜功能。
