Total cavopulmonary connection for surgical treatment of complex congenital heart disease.

Diversion of vena caval flow directly into the pulmonary circulation--total cavopulmonary connection--has been tried at our hospital for two years as an alternative to the modified Fontan procedure for surgical treatment of complex congenital heart disease other than tricuspid atresia in 26 cases with six operative mortalities. The causes of death were low cardiac output in four, uncontrollable paroxysmal supraventricular tachycardia in one and airway obstruction in one. Except for one late death which occurred three months after surgery due to sepsis, the 19 patients who survived the operation were followed up for four to 27 months (mean 15 months). All of them improved clinically (NYHA Class 1). Graded bicycle exercise tests were performed in five of them at three to 12 months after surgery, and their exercise tolerance was comparable to that of patients with an atriopulmonary connection. In conclusion, total cavopulmonary connection is an acceptable alternative to the modified Fontan operation for surgical treatment of complex congenital heart disease, although longer follow-up is necessary.