Results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly / 中华胸心血管外科杂志

ABSTRACT

Objective To evaluate the results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly.Methods From September 2008 to October 2013,34 patients with heterotaxy syndrome underwent 46 cardiac operations at Guangzhou Women and Children Medical Center.22 were male,and 12 female.Mean age at operation was 22 months (4 months to 14 year).There were right atrial isomerism 24 cases,left atrial isomerism 10 cases.Only one had a single VSD,and others were all with complex cardiac anomaly,including complete atrioventricular canal defect 26 cases,tricuspid atresia 4 cases,mitral atresia 1 case,anomalous pulmonary venous connection 18 cases,pulmonary venous stenosis 4 cases,double superior vena cava 17 cases,and interrupted hepatic portion of the inferior vena cava 5 cases.Fontan procedure was performed in 5 patients,bidirectional Glenn in 13,Kawashima in 3,central shunt in 1,atrioventricular valve replacement in 5,and pulmonary artery banding in 5.Results There were 4 died during hospitalization.The follow-up duration was 1 month to 5 years.There were 2 died.The early to middle term mortality was 17.6％ (6/34).In the same period the mortality for 103 patients without heterotaxy syndrome underwent univentricular repair was 4.8％ (5/103).There was significant statistical difference (P ＜ 0.05).Conclusion Right heart bypass operation remains the preferred palliative procedure for patients with heterotaxy syndrome,and the eraly and middle term results were satisfied.