The landscape of congenital heart disease treated with the Ross procedure

The Ross procedure has excellent outcomes in the pediatric population. Some series have reported age- and anatomy-dependent outcomes, but a comprehensive analysis stratified by these variables has not been reported to date. We sought to describe the landscape of congenital heart disease (CHD) treated with the Ross procedure and identify the patients best served by this operation.

Over a total of 30 years, 317 pediatric patients underwent the Ross procedure. These patients were stratified into 4 age groups-neonates (age <31 days; n = 21), infants (age 31 days to 1 year; n = 40), children (age 1-12 years; n = 165), and adolescents (age 13-18 years; n = 91)-and 3 anatomic groups-isolated aortic valve (AV) disease (n = 221), Shone complex or multilevel left ventricular outflow tract (LVOT) obstruction (n = 61), and complex CHD (n = 35). The groups were compared across outcomes.

Neonates and infants had the highest rates of Shone syndrome (P < .001), complex CHD (P < .001), concomitant root enlargement (P < .001), and arch procedures (P < .001). Operative mortality and morbidity were 4.4% (n = 14/317) and 14% (n = 44/317), respectively. Both were higher in neonates and infants (P < .001 for both) but lower for patients with isolated AV disease (P < .001 for both). Ten-year survival and freedom from LVOT reintervention were 92% and 81%, respectively, and were significantly better in patients with isolated AV disease compared to those with complex CHD (P < .001 and P = .005, respectively). In neonates and infants with isolated AV disease, operative mortality was 4% (n = 1/23), morbidity was 9% (n = 2/23), 10-year survival was 85.6%, and 10-year freedom from LVOT reintervention was 88.4%.

Among pediatric patients, those with isolated AV disease are best served with the Ross procedure, regardless of age. Complex CHD is associated with lower survival and increased risk of LVOT reintervention.