小儿完全性肺静脉畸形引流的外科治疗

Correction of total anomalous pulmonary venous connection in children

目的 评判完全性肺静脉畸形引流早期手术矫治的疗效及心上型完全性肺静脉畸形引流各种吻合方法的优缺点。方法 1976～ 1999年共收治完全性肺静脉畸形引流 14 0例 (男 81,女5 9) ,平均年龄 ( 7.13± 6.88)岁。心上型 87例 ( 62 .14 % ) ,心内型 4 4例 ( 3 1.4 3 % ) ,心下型 2例( 1.4 3 % ) ,混合型 7例 ( 5 % )。 2例经右心导管检查证实为重度肺动脉高压 ,未能实施矫治手术 ,余患儿均在全麻、低温、体外循环下行矫治术。心上型的吻合方法包括 :心外法 ( 2 1例 ) ,经上腔静脉与升主动脉间吻合法 ( 3例 ) ,经房间隔缺损吻合法 ( 18例 ) ,心外吻合加经房缺吻合 ( 2例 ) ,经左右心房联合切口吻合法 ( 4 3例 )。结果 术后早期 ( 3 0d)死亡 14例 ( 10 % ) ,其中 3例合并其他复杂的心内畸形 ,均死亡 ,故单纯TAPVC的手术死亡 11例 ,死亡率为 8.0 3 % ( 11/ 13 7)。术后主要并发症为心律失常 60例 ( 4 2 .86% )。结论 完全性肺静脉畸形引流宜尽早手术 ,心上型矫治术吻合口足够大是保证手术成功的关键 ,左右房联合切口入路可确保吻合口足够大 ,但心律失常发生率高 ,经上腔静脉与升主动脉间吻合法可减少心房损伤 ,降低心律失常发生率。

Objective To evaluate the outcome of total correction of total anomalous pulmonary venous connection (TAPVC) and efficacy of operation on supracardic type in infants.Methods From October 1976 to December 1995,140 cases (male 81,female 59) underwent total correction of TAPVC.The mean age was 7.13±6.88 years. The supercardic type accounted for 62.14% (87 patients),intracardiac type 31.43% (44 patients),the infracardiac type 1.43% (2 patients),and the mixed type 5% (7 patients). Most of the patients underwent total correction under CPB with the exception of two cases with severe pulmonary hypertension. The approaches of the supracardiac type repair included:anastomosis between the left atrium and common pulmonary vein (21);anastomosis through enlarged ASD (18);combination of the above two approaches (2);anastomasis of superior vena cava to ascending aorta (3);anastomosis through biatrial incisions (43).Results The early operative mortality was 10% (14/140),and 3 cases died of cardiac complex multiple anomalies.The operative mortality of TAPVC was 7.85% .The operative mortality of infants (below 1 year) was 3.57% (1/28). Another patient (3 month,infracardiac type) died of pulmonary infection 50 days postoperatively. The most common postoperative complication was arrhythmia ( 42.85% ).Conclusions TAPVC should be surgically corrected early. Adequate anastomotic aperture is crucial. The biatrial incision keeps a high incidence of postoperative arrhythmia. Superior vena cava and ascending aorta anastomosis can decrease arrhythmia.