改良Fontan手术治疗复杂先天性心脏病

Therapy for Complex Congenital Heart Disease with Modified Fontan Operation

目的 总结改良Fontan手术治疗复杂先天性心脏病的临床经验.方法 1996年11月～2005年5月,采用改良Fontan手术纠治124例复杂先天性心脏病（病种包括三尖瓣闭锁、单心室、右心室双出口、大动脉错位、肺动脉闭锁、矫正型大动脉转位、右心室发育不良等）患者,手术年龄7.6±5.5岁.常温非体外循环下手术19例,体外循环下手术105例.右心房-肺动脉连接17例,右心房-右心室连接19例,全腔静脉-肺动脉连接术（TCPC）88例.23例行分期手术.结果 术后早期（术后30d）死亡17例（13.7%）,其中行右心房-肺动脉连接者死亡率为23.5%（4/17）,行右心房-右心室连接者死亡率为 15.8%（3/19）,行TCPC者死亡率为11.4%（10/88）,同期预留或术后开窗手术死亡率为14.6%（6/41）,分期手术患者死亡率为 8.7%（2/23）.死亡原因：低心排血量、多器官功能衰竭和心室颤动等.术后早期并发症发生率为16.9%（21/124）,主要为胸腔积液、心律失常、心包积液和低心排血量综合征等.术后随访89例,随访时间6～65个月.远期再住院率6.5%,再手术率0.9%.3例出现再发性胸腔积液,3例出现心包积液,1例出现下腔静脉梗阻,均经相应的治疗后治愈.其余患者心功能恢复好.结论 改良Fontan手术是治疗复杂先天性心脏病中功能性单心室的最佳手术方案;房间隔开窗可明显提高术后早期疗效,减少渗出.

Objective To summarize the clinical experience for complex congenital heart disease treated with modified Fontan operation. Methods From November 1996 to May 2005, 124 patients （male 83, female 41; including tricuspid atresia, single ventricle, double outlet of right ventricle, malposition of great arteries, pulmonary atresia, corrected transposition of great arteries, hypoplastic right-heart syndrome, etc. ） underwent modified Fontan operation at age 7.6 ± 5. 5 years. Non-cardiopulmonary bypass was used in 19 patients, 105 patients with cardiopulmonary bypass. Right atria-pulmonary artery connection were performed in 17 patients, right atriaventricular connection were performed in 19 patients, and total cavopulmonary connection （TCPC） were performed in 88 patients. Staged operation were performed in 23 patients. Results The hospital mortality （30 days postoperative） was 13. 7% （17/124）. The hospital mortality of patients undergone right atria-pulmonary artery connection was 23. 5% （4/17）, patients undergone right atria-ventricular connection was 15. 8% （3/19）, patients undergone TCPC was 11.4% （10/88）, patients undergone operation with fenestration was 14. 6% （6/41）, and the patients undergone staged operation was 8. 7% （2/23）. Low cardiac output syndrome, multiple organ failure, and ventricular fibrillation were the cause of death. Morbidity of complications was 16. 9%（21/124） in early period. Complications consisted of pleural effusion, arrhythmia, pericardial effusion and low cardiac output syndrome, etc. Eighty-nine patients were followed up, follow-up time was from postoperative 6 months to 65 months. Rehospitalization rate was 6.5%, and re-operation rate was 0. 9%. There were pleural effusion in 3 patients, pericardial effusion in 3 patients, and obstruction of inferior vena cava in 1 patient. All patients recovered. Conclusion Modified Fontan operation is an optimal procedure for functional single ventricle, fenestration seems to decrease postoperative pleural effusions.