Surgical Treatment Of Heterotaxy Syndrome With Complex Cardiac Anomaly

Objective(1)To evaluate the results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly.(2)To summarize the results and experience of modified Fontan procedure for patients with heterotaxy syndrome.(3)To summarize the surgical results of patients with heterotaxy syndrome and total anomalous pulmonary venous connection(TAPVC).Methods(1)From September 2008 to December 2017,66 patients with heterotaxy syndrome underwent 101 cardiac operations.48 were male,and 18 female.Mean age at operation was 13.8 months(5 days to 18 year).There were right atrial isomerism 53 cases,left atrial isomerism 13 cases.We performed Fontan in 26 patients,bidirectional Glenn in 23,bilateral bidirectional Glenn in 19,central shunt in 7,atrioventricular valve replacement in 8,and pulmonary artery banding in 8.(2)From September 2008 to December 2017,26 patients with heterotaxy syndrome underwent Fontan precedure.For patients with apicocaval juxtaposition,care must be taken in regard to critical conduit oppression by the ventricle or compression on pulmonary veins.16 were male,and 10 female.Median age at operation was 53.4 months(27 months to 13.5 years).There were right atrial isomerism 20 cases,left atrial isomerism 6 cases.(3)From September 2008 to December 2017,35 patients with single ventricle and TAPVC underwent cardiac operations.The median age and body weight at surgery were 1 year(5 days to 10 years)and 8.2 kg(3.3 to 19.2 kg).The type of TAPVC was supracardiac in 31 patients,infracardiac in 2 patients and mixed in 2 patients.All patients were diagnosed with complex cardiac anomaly,including complete atrioventricular canal defect 25 cases,tricuspid atresia 3 cases,mitral atresia 1 cases,atrioventricular valve regurgitation? moderate 7 cases.Results(1)There were 13 died during hospitalization.The follow-up duration was 9 month to 10 years.There were 4 died.The early to middle term mortality was 27.3%(18/66).(2)In 8 patients with apicocaval juxtaposition,the extracardiac conduit was placed between the inferior vena cava(IVC)and the ipsilateral pulmonary artery behind the ventricle.In the 3 other patients,the conduit was placed between the IVC and the contralateral pulmonary artery.There was hospital mortality.The follow-up duration was 9 months to 9 years.There were 1 died.The mortality was 7.6%(2/26).(3)There were 9 died after initial operation.The cause of dead was low cardiac output syndrome.The follow-up duration was 14 to 130 months.15 patients proceeded to a modified Fontan operation.There were 2 died during follow-up.The overall mortality was 31.4%(11/35).Total Fontan completion was 37.1%.Conclusions(1)Right heart bypass operation remains the preferred palliative procedure for patients with heterotaxy syndrome,and the eraly and middle term results were satisfied.Pulmonary venous stenosis was a risk factor for operative dead.(2)Right heart bypass operation remains the preferred palliative procedure for patients with heterotaxy syndrome,and the eraly and middle term results were satisfied.Modified Fontan procedure in functional single ventricle and apicocaval juxtaposition can be carried out with favorable midterm outcomes.Care must be taken in regard to critical conduit oppression by the ventricle or compression on pulmonary veins.(3)Heterotaxy syndrome with TAP VC was frequently associated with right isomerism and complex cardiac anomaly,and the surgical outcomes remain poor.