[III-JCKAP6-1-4] Surgical results of early stage atrioventricular repair in single ventricle
キーワード：single ventricle, atrioventricular valve repair, Asplenia
IntroductionAtrioventricular regurgitation among single ventricle patients is still a difficult problem. However, we have to face with this issue when the patient is struggling with unstable hemodynamics even though early stage of life. We have reviewed contemporary results of early atrioventricular valve repair.MethodsWe have studied the patients with single ventricle physiology underwent atrioventricular valve repair under 3 months of age between 2008 and 2013.ResultsIn our hospital, 18 patients underwent atrioventricular valve repair before 3 months of age during the study period. 12(66%) patients are diagnoses as unbalanced AVSD, 5(28%) patients are Hypoplastic left heart syndrome, and one patient diagnosed as Pulmonary atresia with hypoplastic Right ventricle. Heterotaxia syndrome was found in 9(50%) patients. Severe atrioventricular valve regurgitation were found in 7(39%), whereas moderate regurgitation were in 12. 12(67%) patients had previous surgery. 6 had bilateral PA banding, 3 had main PA banding, 2 had BT shunt, and one had TAPVC repair before valve surgery. Median age at AV valve repair was 28 days (1-87 days) and median body weight was 2.8kg (2.2-4.0kg). 4(22%) patients had early mortality and 5 had late mortality. 11 patietns(61%) reached bidirectional cavopulmonary shunt, and 5 patients (28%) reached Fontan operation.ConclusionIn this challenging group, late survival was still suboptimal and only 28% of patients reached Fontan procedure in this study.