OBJECTIVETo review the experience of staged total cavopulmonary connection (TCPC) in complex congenital heart diseases.

METHODSFrom June 1998 to March 2008, 22 patients underwent staged TCPC for complex congenital heart diseases. Among them, 9 were univentricular and pulmonary artery valve stenosis; 3 were univentricular and pulmonary artery atresia; 1 was transposition of great arteries, crisscross heart and pulmonary artery valve stenosis; 1 was complete atrioventricular canal defects, left ventricular hypoplasia, pulmonary artery atresia and atrioventricular valvular regurgitation; 1 was complete atrioventricular canal defects, left ventricular hypoplasia, pulmonary artery valve stenosis and atrioventricular valvular regurgitation after Glenn procedure; 1 was mirror image dextrocardia, single ventricle, pulmonary artery atresia, major aortopulmonary collateral arteries (MAPCAs) and right pulmonary arteriovenous fistula after Glenn procedure; 4 were tricuspid atresia and pulmonary artery valve stenosis; 1 was tricuspid atresia and pulmonary atresia; 1 was mirror image dextrocardia, double-outlet of right ventricle, left ventricular hypoplasia, pulmonary artery valve stenosis, tricuspid incompetence, and MAPCAs. Among them, 5 patients received systemic-to-pulmonary artery shunt, bidirectional Glenn procedure and TCPC. Seventeen patients received bidirectional Glenn procedure, the mean age was (5.9+/-4.4) years old. Pulmonary artery pressure pre-Glenn procedure was 17 to 20 mm Hg (1 mm Hg=0.133 kPa). Atrioventricular valve incompetence in 3 patients. Nakata index was less than 200 mm2/m2 in 4 patients before the first stage operation. The age of TCPC procedure was (9.6+/-4.9) years old, the interval time was (3.7+/-1.2) years.

RESULTSThere was one in-hospital death, the mortality was 4.5%. The patient with univentricular and pulmonary atresia, received systemic-to-pulmonary artery shunt, bidirectional Glenn procedure and TCPC and died of pneumorrhagia. Other patients were recovered well, postoperative central venous pressure was 12 to 18 mm Hg, percutaneous oxygen saturation was 90% to 96%. The cardiac function were in NYHA class I to II.

CONCLUSIONSThe staged TCPC was a good procedure in high-risk Fontan candidates. The results were satisfactory for those patients. This staged strategy may extend the operative indications for the Fontan procedure.