Repair of atrioventricular canal malformation in the first year of life.

Disappointing results with pulmonary artery banding and subsequent correction led to the decision in 1977 that all infants presenting to our hospital with atrioventricular (AV) canal and evidence of severe heart failure, lack of growth, or pulmonary hypertension should have early operative correction. Since that time 24 consecutive infants have undergone repair. All had refractory heart failure. Average age at operation was 18 weeks (3 to 38) and average weight was 4.3 kg (2.3 to 6.4). Only four patients were older than 6 months of age at operation. Preoperative peak pulmonary artery pressure was 81 +/- 3.3 mm Hg, which was equal to systemic arterial pressure in all cases. Mean pulmonary-to-systemic resistance ratio was 0.28 +/- 0.05. five patients had moderate mitral regurgitation and five had a ductus arteriosus. Three had significant associated malformations. Profound hypothermia and circulatory arrest were utilized in all patients. Common AV valve tissue was divided and valvular integrity was ensured by resuspension to a single Dacron patch which closed both the atrial and ventricular defects. Operative death occurred in two patients (8%) both with associated defects (one with total anomalous pulmonary venous connection and the other with coarctation). One late death occurred in a patient with associated partial anomalous pulmonary venous connection, and one patient has had a pacemaker implanted. Survivors have been followed for 7 to 60 months. All patients are growing at an increased rate postoperatively. All cardiac medications have been discontinued in 16 of 21 patients. Operative repair of complete atrioventricular canal can be performed in infancy with low operative and late death rates and will relieve signs and symptoms of heart failure and allow more normal growth and development. On the basis of this experience, it appears unnecessary to delay operative correction with the known increased risk of the development of pulmonary hypertension.