First experience of staged treatment of an infant with heterotaxy syndrome with total anomalous pulmonary vein drainage combined with a single ventricular heart and pulmonary atresia

The combination of monoventricular congenital heart disease with total anomalous pulmonary vein drainage and pulmonary atresia, is a rare pathology and characterized by an extremely severe course with the highest mortality. In the presented work we described a clinical case of the first and successful experience of two-stage treatment of an infant with total anomalous pulmonary vein drainage combined with single ventricular heart disease and pulmonary atresia in the Republic of Kazakhstan. For total anomalous pulmonary vein drainage correction we used a sutureless technique — primary sutureless repair, which is used in our center as a primary one. Due to severe anatomy of the defect, we used unusual area of the distal end of the central systemic-pulmonary artery anastomoses during primary surgery at the mouth of the severed patent ductus arteriosus in the area of pulmonary artery bifurcation, where it is associated with high surgical risks. Taking into account the presence of an additional obstacle to pulmonary blood flow in the form of left branch pulmonary artery orifice stenosis, we performed one-stage dilatation plasty of the pulmonary artery branch bifurcation with autopericardium. During the repeated surgery we faced such a serious complication as aortic damage for the first time, thanks to emergency actions it did not turn out to be fatal. We also managed to perform the most preferable dilation of hypoplastic branches of the pulmonary artery by plasty with the own tissues of the superior vena cava during the formation of Glenn-anastomosis.