The Ross procedure in children: a systematic review

Background: The Ross procedure involves autograft transplantation of the native pulmonary valve into the aortic position and reconstruction of the right ventricular outflow tract (RVOT) with a homograft. The operation offers the advantages of a native valve with excellent hemodynamic performance, the avoidance of anticoagulation, and growth potential. Conversely, the operation is technically demanding and imposes the risk of turning single-valve disease into double-valve disease. This systematic review reports outcomes of pediatric patients undergoing the Ross procedure.
Methods: An electronic search identified studies reporting outcomes on pediatric patients (mean age <18 years, max age <21 years) undergoing the Ross procedure. Long-term outcomes, including early mortality, late mortality, sudden unexpected unexplained death, reoperation due to failure of the pulmonary autograft or RVOT reconstruction, thromboembolic events, bleeding events, and endocarditis-related complications, were evaluated.
Results: Upon review of 2,035 publications, 30 studies and 3,156 pediatric patients were included. Patients had a median age of 9.5 years and median follow-up period of 5.7 years. Early mortality rates varied from 0.0 to 17.0% and were increased in the neonatal population. Late mortality rates were much lower (0.04–1.83 %/year). Reoperation due to pulmonary autograft failure occurred at rates of 0.37–2.81 %/year and reoperation due to RVOT reconstruction failure was required at rates of 0.34–4.76 %/year. Thromboembolic, bleeding, and endocarditis events were reported to occur at rates of 0.00–0.58, 0.00–0.39, and 0.00–1.68 %/year, respectively.
Conclusions: The Ross operation offers a durable aortic valve replacement (AVR) option in the pediatric population that offers favorable survival, excellent hemodynamics, growth potential, decreased risk of complications, and avoidance of anticoagulation. Larger multi-institutional registries focusing on pediatric patients are necessary to provide more robust evidence to further support use of the Ross procedure in this population.