Objective: Controversy persists with regard to the treatment of patients with aortic atresia. Staged reconstructive operations and primary transplantation have been advocated as treatment strategies, but in many instances no treatment is undertaken. A multi-institutional study was undertaken for the purpose of characterizing this challenging patient group, comparing the prevalence and outcomes of the various treatment strategies, and identifying potential predictors of success or failure with each.
Methods and results: A total of 323 neonates with aortic atresia were entered into a 21-institution prospective, nonrandomized study between January 1, 1994, and January 1, 1997. Three protocols were used, nonexclusively in many institutions: (1) staged reconstructive surgery with initial palliation by a Norwood procedure and eventual Fontan operation, (2) heart transplantation as initial definitive therapy, and (3) nonsurgical management. Analysis was based on initial protocol assignment: staged reconstructive surgery in 253 patients, heart transplantation in 49 patients, and nonsurgical management in 21 patients. For all patients initially entered into the 2 surgical treatment protocols, survival at 1, 3, 12, 24, and 36 months after entry was 67%, 59%, 52%, 51%, and 50%, respectively. A multivariable analysis found incremental risk factors for death at any time after entry to be lower birth weight (P=.04), associated noncardiac anomaly (P=.007), and entry into the nonsurgical protocol (P < .0001) or the staged reconstructive surgery protocol (P=.03). Four institutions had higher survival statistics; 2 used a heart transplantation protocol and 2 used a staged reconstructive surgery protocol. For the 113 patients treated at these 4 institutions, survival at 1, 3, 12, 24, and 36 months after entry was 77%, 70%, 64%, 62%, and 61%, respectively. Survival among the 4 institutions was similar (P=0.1).
Conclusions: Among patients with aortic atresia, other features of cardiac structure including aortic size, degree of left ventricular hypoplasia, and degree of mitral hypoplasia or atresia are not predictive of survival from 2 surgical protocols. The highest survival was achieved with either treatment strategy at institutions strongly committed to the use of one or the other surgical management protocol.