Our patient was born with a ventricular septal defect, atrial septal defect, persistent ductus arteriosus, inferior vein cava interruption, pulmonary stenosis, and a hypoplasic tricuspid ring with valve stenosis. At the age of 16 months she underwent surgical correction consisting of closure of both septal defects, ligature of the arterial duct, and pulmonary valvotomy with infundibular resection. The tricuspid valve was left unrepaired. At the age of 6 she developed PLE. Cardiac catheterization revealed significant stenosis of the tricuspid valve caused by hypoplasic ring. She then underwent surgical palliation consisting of a bidirectional Glenn but her PLE was not affected. Afterwards, heparin therapy was started and protein losing decreased with a dose of 7500 Ul/m2/day.

We certainly proved dose–response efficacy of heparin therapy, but heparin was not totally curative. Furthermore heparin caused osteoporosis with subsequent pathological fractures as a side effect. When she was 8 years old, we observed that the gradient through the tricuspid stenosis had increased. The a mean right atrial venous pressure was 18 mmHg by