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The development of systemic collateral veins after palliative surgery in children with univentricular circulation is a common complication, however, manifestation as late as 10 years postoperatively is rare. Massive systemic to hepatic venous collaterals developed in a 14-year-old girl with univentricular heart, situs inversus atriovisceralis and hemiazygos continuity to the left-sided superior vena cava, 10 years after Kawashima operation. The resulting azygoportal shunt had led to a progressive systemic desaturation and reduction in ventricular function. Interventional occlusion was supposed to be risky for renal failure due to potential closure of the renal vein so that surgical closure was performed. The saturation persistently increased from 65% to more than 85% postoperatively.

Late desaturation due to collateral veins 10 years after total cavopulmonary shunt in left atrial isomerism: surgical closure