Echocardiographic Findings Associated with Transplantation-Free Survival and Left Ventricular Systolic Function at Midterm Follow-Up after Ross Procedure in Infants with Critical Aortic Stenosis

Abstract

The Ross operation is an important option for children with critical aortic stenosis with residual disease, but operation in infancy is associated with significant morbidity and mortality. The aim of this study was to evaluate echocardiographic correlates of transplantation-free survival, reintervention, and left ventricular (LV) function in midterm follow-up. This retrospective, single-center study included all infants with critical aortic stenosis who underwent Ross by 1year of age from January 2000 to September 2018. Serial echocardiograms were analyzed for LV ejection fraction (LVEF) and systolic and diastolic longitudinal strain. The primary outcome was mortality or transplantation; secondary outcomes were reintervention and abnormal LVEF (≤55%). Among 40 infants (30 male [75%]; median age at Ross, 51days) with median follow-up duration of 3.3years (interquartile range, 1.0-9.4years), the primary outcome was met in 11 (28%). Rates of transplantation-free survival was 79%, 77%, and 69% at 1, 5, and 10years after Ross. Predictors of transplantation or death included neonatal surgery, cross-clamp time, and preoperative left atrial dilatation and lower LVEF. Median freedom from reintervention was 7.1years after Ross, with no identified associations. LV longitudinal strain improved 1year after Ross (-21.1±3.8% vs -17.4±5.1%, P=.02), although LVEF did not reach significance. Lower LVEF at 1year was related to pre-Ross left atrial dilatation (P=.02), abnormal LVEF (P=.04), and lower early diastolic longitudinal strain rate (P=.03). LVEF remained stable3years after Ross. Both transplantation-free survival and normalization of LV function after Ross in infancy are associated with preoperative LV systolic and diastolic measures, highlighting the prognostic value of echocardiography in this population. Further data are necessary in a larger, multicenter cohort to allow more precise risk stratification.