Medical Results Of Total Cavcopulmonary Connection In Patients With Heterotaxy Syndrome And Researches Of Computational Fluid Dynamics

Objective:To summarize the cardiac-vascular abnormalities and clinical results in patients with Fontan operation for heterotaxy syndrome, and evaluate the variation of the postoperative hemodynamic by computational fluid dynamics. Method:Medical records are retrospectively reviewed in all 81 patients who underwent the Fontan operation with heterotaxy syndrome from September 2008 to September 2013. 49 patients are male and 32 are female, with an average age of 4.48±2.07 years, weight of 15.66±4.32 kg and preoperative room air saturation of 79.20±10.06%. The staged Fontan procedure was applied in 63 patients and one stage in 18. Operation strategies were included intra/extracardiac conduit(IE, n=19), lateral tunnel(LT, n=14)and extracardiac conduit(ECC, n=48),with an average cardiopulmonary bypass time of 130.17±40.38 minutes and an average aortic occlusion time of 67.92±24.08 minutes. Both a right atrial isomerism and left atrial isomerism patient were undertaken the research of computational fluid dynamics. Results : There are 70 right atrial isomerism and 11 left atrial isomerism patients. 95.7% patients of right atrial isomerism are in accordance with asplenia and 90.9% patients in accordance with polysplenia. Right atrial isomerism is characterized of bilateral superior vena cava, complete atrioventricular canal defect and single ventricle of right dominant and left atrial isomerism is characterized of interrupted inferior vena cava. All of conclusions above are confirmed by Meta analysis. There are 15(18.5%)early deaths and all patients are with a median CICU stay of 5(1~27)days and a median ventilator time of 10.70(2.5~551.0)hours postoperatively. Complications are included low cardiac output in 15(18.5%), hepatic insufficiency in 35(43.2%), renal insufficiency in 55(61.7%)and arrhythmia in 41(50.6%) patients, and 28 patients required drug intervention against arrhythmia. The median length of pleural drainage is 11(2~46)days. Multiple logistic regression analysis confirmed that intermediate or severe atrioventricular valve regurgitation, asplenia, anomalous pulmonary venous drainage, aorta pulmonary collateral arteries and pulmonary arterial hypertension were risk factors for postoperative deaths and main complications. The median follow-up time was 33(4~90)months in 66 patients including mid-term deaths in 4 and reoperations in 4 patients. Kaplan-Meier analysis estimated that 5-year morbidity was 72%. Researches of computational fluid dynamics pointed out that both right atrial isomerism and left atrial isomerism have stable hemodynamics, with normal variations of energy loss and ratio of flow between bialateral pulmonary arteries. But turbulence was seen near the trunk pulmonary both in the right atrial isomerism and left isomerism patient. Conclusion:Heterotaxy syndrome is a majority of right atrial isomerism and regular patterns of cardiovascular anatomic deformities were observed based on the trend of symmetry heterotaxia. The Fontan operation is the main strategy for patients with heterotaxy syndrome and key to improving the therapeutic effect is the management of risk factors and access to strict indications of the operation. The extracardiac conduit Fontan is the first choice for these patients and the surgical intervention for patients with atrioventricular valve regurgitation may lead to good results. The technology of computational fluid dynamics is well performed in the observation of postoperative hemodynamics in patients with heterotaxy syndrome, calculated values of which may contribute to the design of individual operation.