Total cavopulmonary connection with innovative combined lateral tunnel and extracardiac conduit in heterotaxy syndrome

Total cavopulmonary connection remains a challenging procedure when treating heterotaxy syndrome patients with a widely separate drainage of the inferior vena cava and the hepatic vein into the common atrium. We trimmed a Gore-Tex tube graft (W.L. Gore & Associates, Flagstaff, AZ) to form a skirt to cover both openings of the inferior vena cava and the hepatic vein. This tubular structure pierces the atrium and travels outside the heart, and then reaches the inferior side of the pulmonary artery to avoid pulmonary vein obstruction. We then sutured the pierced atriotomy margin to the conduit. This innovative procedure has been shown to accommodate the widely separated hepatic vein drainage with a promising outcome.