TOTAL CAVOPULMONARY CONNECTION IN PATIENTS WITH FUNCTIONAL SINGLE VENTRICLE

Nineteen children aged between 1.5 and 9.8 years (10 patients younger than 4 years) underwent total cavopulmonary connection (TCPC) for complex congenital heart defects other than tricuspid atresia. Diagnoses included double inlet left ventricle (n = 11), transposition of the great arteries with a large ventricular septal defect (n = 4), double outlet right ventricle (n = 2), congenitally corrected transposition of the great arteries with a large ventricular septal defect (n = 1) and criss-cross heart (n = 1). Two patients had situs ambiguous with polysplenia syndrome and four patients had anomalous systemic venous return. Thirty-six palliative procedures were performed in 15 patients before TCPC. The patients were selected according to the selection criteria of Choussat and Fontan. Four patients (21 %) died between the 1st and 12th postoperative day because of low cardiac output syndrome (n = 2), supraventricular tachycardia unresponsive to treatment (n = 1), and cerebral edema (n = 1). In one patient take down of TCPC was performed for persistent low cardiac output syndrome. Four of these five patients were younger than 4 years old and had one or more additional risk factors. Postoperative complications were persistent pleural or pericardial effusions (n = 4), transient supraventricular tachycardia (n = 2), and low cardiac output syndrome (n = 1). There was no late death. Follow-up was available in 12 patients. At a mean follow-up of 15 +/- 9 months, nine patients are in NYHA-functional class I, two patients in class II, and one patient in class III. Postoperative cardiac catheterization revealed satisfactory results in seven of eight patients. In three patients interatrial defects after fenestration of the intraatrial patch were closed with a Rashkind umbrella device. Our results demonstrate that TCPC can be successfully performed in various complex heart defects with functional single ventricle.