The results of the Fontan procedure in patients with visceroatrial heterotaxy syndrome and complex cardiac anomalies

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. Key words: Heart defects,congenital; Fontan procedure visceroatrial; Heterotaxy syndrome