Severe protein losing enteropathy occurred in 3 of our 128 patients undergoing the Fontan procedure in the period of 1982 to 1994. The complication occurred 4, 4.5 and 5 years after the procedure, respectively. One patient had double inlet left ventricle while the other two had tricuspid atresia. All had initially undergone an atrio-pulmonary connection, and the duration of the enteropathy was 12, 13,5 and 15 months. All patients were in functional class IV of the New York Heart Association. The presence of protein losing enteropathy was confirmed by determining levels of fecal alfa-1 antitrypsin, enteric clearance of 51 Cromo labeled albumin, and endoscopy with histopathological study of the small bowel.
They underwent further surgery to convert the atrio-pulmonary to a cavo-pulmonary anastomosis which excluded all the hepatic veins. A bovine pericardial baffle was sutured in the inferior caval vein so as to leave the hepatic venous flow draining to the right atrium and thence through an atrial septal defect to the left atrium and systemic circulation. The inferior caval venous flow was directed through an intra-atrial tunnel to the right pulmonary artery, and the superior caval vein was anastomosed to the superior aspect of the right pulmonary artery, thus completing the total cavo-pulmonary anastomosis with exclusion of the portal venous flow.
Postoperatively, there was total regression of the clinical and laboratory findings. All patients are now in functional class I or II of the New York Heart Association. They have mild to moderate cyanosis up to three and a half years from the reoperation. We recommend the technique as an alternative for patients with severe protein losing enteropathy occurring after the Fontan procedure.