Total cavopulmonary connection--early results

Presently total cavopulmonary connection is used as an alternative in complex congenital disorders requiring atriopulmonary connection. From January 1989 till November, 1992, a total of 18 cases underwent total cavopulmonary connection. There were 8 males and 10 females. The mean age at operation was 92 months (range 5 to 504 months). The average weight was 19.6 kg (range 5.25 to 42 kg). Diagnoses included: tricuspid atresia (6); univentricular heart (7); pulmonary atresia with intact interventricular septum (1); double outlet right ventricle, pulmonary stenosis with uncommitted ventricular septal defect (2); corrected transposition of the great arteries with multiple ventricular septal defects (1) and complete atrioventricular septal defect with double outlet right ventricle (1). The mean duration of ventilatory and inotropic support was 127 hours (range 12 to 528 hours) and 140 hours (range 24 to 528 hours) respectively. The average duration of hospital stay was 17.9 days (range 5 to 30 days). There was no intra-operative mortality. Early mortality was 22.2% (4/18). Pre-operative diagnoses of patients who died were: tricuspid atresia (1), univentricular heart (1), complete atrio-ventricular septal defect with double outlet right ventricle (1) and double outlet right ventricle, pulmonary stenosis with uncommitted ventricular septal defect (1). The causes of death were: low-output syndrome (3) and septicemia (1). Although these initial results with total cavopulmonary connection suggest a lower early mortality compared to other modifications of the Fontan principle, long-term follow-up would be necessary to assess the late implications of this procedure.