Tricuspid atresia presenting in infancy. Survival and suitability for the Fontan operation.

BACKGROUND: The Fontan operation is the usual goal of therapy for children with tricuspid atresia. The influences of morphology and different management strategies on survival and subsequent suitability for this procedure are crucial but unstudied in an unselected population during the Fontan era.

METHODS AND RESULTS: The fates of 237 consecutive infants with tricuspid atresia were reviewed (1972-1987; median follow-up, 8.0 years). Overall actuarial survival was 72% at 1 year, 53% at 5 years, and 46% at 10 years. Univariate risk factor analysis established that discordant ventriculoarterial connections (24% of the group; relative risk, 2.7), pulmonary atresia (14%, 2.3), aortic arch obstruction (7%, 2.9), and subaortic stenosis (8%, 4.2) were associated with poor survival, whereas pulmonary stenosis (60%, 0.52), balanced pulmonary blood flow (9%, 0.25), and older age at presentation (33%, 0.42) were beneficial. Multivariate analysis allowed the creation of predictive patient-specific survival curves and two additive indexes. Survival was worse for patients who underwent banding of the pulmonary trunk with aortic arch repair than for other individual palliative procedures (p < 0.001). On retrospective review, 204 patients (86%) were judged suitable for a future Fontan procedure at presentation. However, 99 (48%) of these are known to have died before a Fontan operation or became unsuitable for such surgery during follow-up, mostly because of death after palliative surgery (23 patients, 11%), sudden death (18 patients, 9%), and new adverse features (32 patients, 16%) such as subaortic stenosis, pulmonary arterial distortion, and ventricular dysfunction.

CONCLUSIONS: Management in infancy must aim to ensure survival and maintain suitability for a Fontan-type operation. The accumulating incidence of adverse events with increasing age would argue in favor of undertaking definitive surgery in early childhood in most patients.