Objective To assess the results of the Fontan procedure in patients with visceroatrial heterotaxy syndrome and complex cardiac anomalies.Methods From April 2002 through December 2010,25 patients (male 10,female 15) had undergone the Fontan procedure for heterotaxy syndrome or atrial isomerism and complex congenital heart disease 28 times.Median age at operation was (9.1 ± 5.5) years (2 to 18 years).Heterotaxy syndrome were associated with right atrial isomerism (n =18) or left atrial isomerism (n =7),asplenia (n =13) or polysplenia (n =7),double inlet of left ventricle (n =15),double inlet and outlet of left ventricle (n =5),double outlet right ventricle with pulmonary atresia (n =2) and with pulmonary stenosis (n =1),tricuspid or mitral atresia (n =2).A bidirectional cavopulmonary shunt was performed in 8 patients (bilateral in 3 patients).A cavopulmonary shunt placement,so-called Kawashima operation,was performed in 4 patients.An extracardiac conduit Fontan connection was pefformed in 15 patients and intracardiac lateral tunnel Fontan connection in one patient.Results 2 patients died in hospital caused by ventricular failure.Five patients developed early postoperative atrial arrhythmias and 2 patients had sinus node dysfunction.Mean arterial oxygen saturation at discharge was 0.86 ± 0.07 (range,0.78 to 1.00).Follow-up (range,0.5 to 7 years) was available on 15 patients.Mean arterial oxygen saturation was 0.82 ±0.08 (range,0.68 to 0.97).Ventricular function was normal in 13 patients (EF range,0.50 to 0.66) and depressed in 2 patients.Four patients had a junctional rhythm.Conclusion The Fontan procedure was still the main procedure for patients with visceroatrial heterotaxy syndrome and complex cardiac anomalies,which can reach satisfactory early and medium-term results.The choice of Fontan procedure,extracardiac conduit Fontan connection,aggressive treatment of concomitant malformations were essential to improve the outcomes.%目的 评价Fontan类手术治疗心房异构、内脏异位合并心脏畸形的效果.方法 2002年4月至2010年12月,25例心房异构、内脏异位合并心脏畸形共行手术治疗28次.男10例,女15例,年龄2～18岁,平均(9.1±5.5)岁.右心房异构18例,左心房异构7例.左心室双入口合并肺动脉狭窄15例,左心室双入口和双出口合并肺动脉狭窄5例,右心室双出口合并肺动脉闭锁2例,合并肺动脉狭窄1例,二尖瓣和三尖瓣闭锁合并肺动脉狭窄各1例.Fontan手术16例次,其中15例次行心外管道Fontan手术,1例次行心房内侧隧道Fontan手术;双向腔肺分流术8例次,3例次为双上腔静脉肺动脉分流;全腔静脉肺动脉分流4例次(Kawashima手术).结果 术后早期死亡2例,均为术后严重低心排血量综合征.术后室上性心动过速5例,结性心律2例.术后经皮血氧饱和度0.78 ～ 1.00,平均0.86±0.07.随访6个月以上者15例,最长7年,经皮血氧饱和度0.68 ～0.97,平均0.82±0.08;心室射血分数＞0.55者13例,＜0.50者2例;结性心律4例.结论 Fontan类手术是治疗心房异构、内脏异位合并心内复杂畸形的主要手术方式,术后早、中期效果较好.选择Fontan手术的术式,特别是心外管道,妥善修复心内合并畸形是提高效果的关键.
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