Results of total cavopulmonary connection in the treatment of patients with a functional single ventricle.

Total cavopulmonary connection was proposed as a modification of the Fontan procedure that might have greater benefits than previous methods. To assess this procedure we reviewed case histories of 38 patients (aged 17 months to 30 years) who underwent Fontan procedures with cavopulmonary anastomoses between January 1987 and December 1989. The group included 32 patients with univentricular heart, 2 with pulmonary atresia and intact ventricular septum, 3 with tricuspid atresia, and 1 with hypoplastic left heart syndrome. One or more previous palliative procedures were performed in 34 patients, including 19 systemic-pulmonary shunts, 16 pulmonary artery bandings, 7 atrial septectomies/septostomies, 7 Glenn shunts, and 1 patent ductus arteriosus ligation. Preoperative hemodynamics showed a pulmonary artery pressure of 12 mm Hg (range 6 to 22 mm Hg), pulmonary-systemic flow ratio of 1.6 (range 0.37 to 3.0), left ventricular end-diastolic pressure 9 mm Hg (range 3 to 15 mm Hg), and systemic arterial oxygen saturation of 82% (range 67% to 94%). Concomitant with cavopulmonary connection, 13 patients underwent additional procedures, including 9 atrioventricular valve annuloplasties, 4 Damus-Stansel-Kaye procedures, and 2 resections of subaortic membranes. Modifying the Fontan procedure in this fashion was particularly useful in the management of 2 patients with pulmonary atresia and intact ventricular septum who had right ventricular-dependent coronary blood flow. Cavopulmonary anastomosis and atrial septectomy were performed in both patients, with resultant inflow of oxygenated blood to the right ventricle and coronary arteries. Excellent postoperative results were noted in each. Postextubation hemodynamics for the entire group included a mean right atrial pressure of 13 mm Hg (range 11 to 17 mm Hg), a mean left atrial pressure of 6 mm Hg (range 3 to 12 mm Hg), and a room air oxygen saturation of 96% (range 92% to 98%). Seven patients had pleural effusions, 3 required postoperative pacemaker placement, and 2 required reoperation for tamponade. A venous assist device was required in one patient on the second postoperative day, but the patient was weaned successfully within 24 hours. One early death (2.6%) occurred in a patient who had intractable ventricular fibrillation 2 days after operation. There was one late cardiac death (2.7%) caused by ventricular failure and one late noncardiac death. These results demonstrate that total cavopulmonary connection provides excellent early definitive treatment, with low morbidity and mortality, for a variety of complex congenital heart lesions.