[Percutaneous closure of veno-venous fistulae after total cavopulmonary derivation: a case report]

The authors report the case of a boy with a complex congenital heart malformation with a dextro-isomerism or Ivemark's syndrome. After several palliative procedures, a total cavopulmonary derivation was performed at the age of 11 years with implantation of a fenestrated tube between the inferior vena cava and the right pulmonary artery. The fenestration was closed two years later. At about 15 years of age, progressive systemic desaturation was observed; cardiac catheterisation with angiography showed a large number of intra-hepatic veno-venous collaterals draining into a large left suprahepatic vein which drained into the right atrium connected to the systemic ventricle. The largest fistula was embolised with a 20 mm Amplatzer Duct Occluder and two others with 14 x 12 and 10 x 8 Amplatzer Duct Occluders. The saturation rose from 75% to 94%. After 6 months' follow-up, the child is asymptomatic and the systemic saturation is 90%.