Pediatric Heart Network: Single Ventricle Reconstruction Extension Study (SVR III)

This multi-center study of the NHLBI-supported Pediatric Heart Network, Long-term Outcomes of Children with HLHS and the impact of Norwood Shunt Type (SVR-III) is designed to determine if shunt type at the time of Norwood operation is associated with long-term differences in cardiac function, survival or contributors to quality of life and to characterize long-term outcomes and determine risk factors other than shunt type for adverse long term outcomes in children with hypoplastic left heart syndrome (HLHS) and other related single ventricle anomalies. Dr. Newburger is the national co-PI, together with Dr. Caren Goldberg at University of Michigan. The primary aim is to compare ventricular function between the two groups and the primary outcome measure is right ventricular ejection fraction as measured by cardiac magnetic resonance (CMR). Secondary aims include comparison between the two shunt groups for transplant free survival, arrhythmia burden, exercise tolerance, and, of special importance to this population, neurodevelopmental outcomes. Through the SVR III study we will continue the collection of DNA specimens from this cohort which will be available for future genetic and biomarker studies.Eligible members of the SVR cohort, transplant-free survivors with single ventricle physiology with or without a Fontan operation will be invited to an SVR clinical site to return for in-person follow-up at 11 years + 1 year. In addition medical history will be reviewed annually for each participant through the SVR III study period. For those patients who have undergone heart transplant only vital status will be collected.