[Total cavopulmonary connection in complex heart defects with a single functional 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.(ABSTRACT TRUNCATED AT 250 WORDS)