Repair of congenital cardiac lesions during the first week of life

Repair of congenital cardiac defects in the neonatal period has been associated with a high operative mortality thought to be due to the adverse effects of cardiopulmonary bypass. This has prompted the use of closed palliation in such patient. Between 1975 and 1981, 195 of 988 patients (20%) operated in the first year of life were critically ill neonates ages 1-7 days. Fifty-six underwent repair using cardiopulmonary bypass. These included 29 operations at the arterial level, five for aortic stenosis, 19 for pulmonary stenosis and five for transposition of the great arteries using the switch procedure; 25 at the atrial level, eight for transposition of the great arteries, 16 for total anomalous pulmonary venous connection, and one for combined total anomalous pulmonary venous connection and anomalous systemic venous connection; and two at the ventricular level, one for tetralogy of Fallot and one for double-outlet right ventricle. The hospital survival rate in this group was 91%. Palliations using cardiopulmonary bypass were performed in an additional 27 patients, including right ventricular outflow tract patches in 20. The survival rate in this group was 73%. The survival rate was 100% for closed repairs and 76% for palliations. These results demonstrate that cardiopulmonary bypass per se is not a determinant of survival in the neonate; an effective repair combined with proper diagnostic techniques and postoperative management are probably the critical factors.