Cor triatriatum is an uncommon cardiac anomaly occurring in 0.1% of children with congenital heart disease. We reviewed our results from 20 patients presenting with cor triatriatum in the last 20 years (from March 1983 to August 2002). Mean age at repair was 20 months (1-132 months). During a mean follow-up period of 31 months (2-156 months) there were no deaths. All patients were in sinus rhythm except one patient who needed pacemaker insertion one week after repair. A total of 14 patients were asymptomatic and 6 were in New York Heart Association Class I. Cor triatriatum is amenable to surgical repair with excellent results when diagnosed early, and when not associated with other severe cardiac anomalies.

Results of the two-stage arterial switch operation in 49 patients with transposition of the great arteries, performed between January 1995 and September 2000, were reviewed retrospectively. Twenty-one patients had a ventricular septal defect. Anatomical correction was carried out 21.89 +/- 9.86 months after pulmonary artery banding, with or without a modified Blalock-Taussig shunt. Hospital mortality was 8% (4 patients). During follow-up of 30.12 +/- 14.38 months, there was 1 late death and 1 patient required reoperation for pseudoaneurysm of the ascending aorta. Actuarial survival and freedom from reoperation at 5 years were 90% and 97%, respectively. Late anatomic correction (> 6 months) after the preliminary procedure can be performed with an acceptable mortality and morbidity, but undue delay may lead to left ventricular dysfunction, arrhythmias, and new aortic valve regurgitation or subaortic stenosis.

Primary repair of complete atrioventricular canal in patients who present beyond one year of age carries a high mortality. Between January 1995 and February 2000, 16 patients aged 8 to 24 months (mean, 14.5 months) received pulmonary artery banding at presentation and underwent total correction at 24 to 96 months old (mean, 41.9 months). There was one hospital death (mortality, 6.25%). During a mean follow-up of 10.2 months (range, 6 to 28 months), there was no late death, 13 of the 15 survivors (87%) were in New York Heart Association functional class I, and 2 (13%) were in class III. In patients with complete atrioventricular canal who present late with severe reactive pulmonary hypertension, banding followed by complete repair reduces the risk associated with primary repair.

A child diagnosed with transposition of great arteries, multiple (Swiss cheese) ventricular septal defects, and a small right ventricle underwent pulmonary artery banding and patent ductus arteriosus ligation at the age of six months. At the age of three years bidirectional cavopulmonary shunt was performed as a first stage for univentricular repair. However, the patient was lost follow-up for four years, following which further evaluation showed that the right ventricle was reasonably adequate to support pulmonary circulation if the ventricular septal defects (VSDs) were closed using percutaneous techniques. Four VSDs were then closed using Amplatzer devices (AGA Medical Corporation, Plymouth, MN, USA). At the age of eight years she underwent complex biventricular repair in the form of arterial switch, closure of atrial septal defect, take down of Glenn shunt, and reanastomosis of the distal end of the superior vena cava to the distal superior vena cava stump on the right atrium. One year later the patient is alive and well. In conclusion; biventricular repair may be considered before completion of Fontan whenever cardiac anatomy allows.