Faculty Collaboration Database

The pediatric heart network's study on long-term outcomes of children with HLHS and the impact of Norwood Shunt type in the single ventricle reconstruction trial cohort (SVRIII): Design and adaptations. Am Heart J 2022 Dec;254:216-227

Date

09/18/2022

Pubmed ID

36115392

DOI

10.1016/j.ahj.2022.09.005

Scopus ID

2-s2.0-85139857587 (requires institutional sign-in at Scopus site)   8 Citations

Abstract

BACKGROUND: The Single Ventricle Reconstruction (SVR) Trial was the first randomized clinical trial of a surgical approach for treatment of congenital heart disease. Infants with hypoplastic left heart syndrome (HLHS) and other single right ventricle (RV) anomalies were randomized to a modified Blalock Taussig Thomas shunt (mBTTS) or a right-ventricular-to-pulmonary-artery shunt (RVPAS) at the time of the Norwood procedure. The aim of the Long-term Outcomes of Children with HLHS and the Impact of Norwood Shunt Type (SVR III) study is to compare early adolescent outcomes including measures of cardiac function, transplant-free survival, and neurodevelopment, between those who received a mBTTS and those who received an RVPAS.

METHODS: Transplant-free survivors of the SVR cohort were enrolled at 10 to 15 years of age for multifaceted in-person evaluation of cardiac function (cardiac magnetic resonance [CMR], echocardiogram and exercise test) and neurodevelopmental evaluation. Right ventricular ejection fraction measured by CMR served as the primary outcome. Development of arrhythmias, protein losing enteropathy, and other comorbidities were assessed through annual medical history interview. Through the course of SVR III, protocol modifications to engage SVR trial participants were designed to enhance recruitment and retention.

CONCLUSIONS: Evaluation of long-term outcomes will provide important data to inform decisions about the shunt type placed at the Norwood operation and will improve the understanding of cardiovascular and neurodevelopmental outcomes for early adolescents with HLHS.

Author List

Goldberg CS, Gaynor JW, Mahle WT, Ravishankar C, Frommelt P, Ilardi D, Bellinger D, Paridon S, Taylor M, Hill KD, Minich LL, Schwartz S, Afton K, Lamberti M, Trachtenberg FL, Gongwer R, Atz A, Burns KM, Chowdhury S, Cnota J, Detterich J, Frommelt M, Jacobs JP, Miller TA, Ohye RG, Pizarro C, Shah A, Walters P, Newburger JW, Pediatric Heart Network Investigators

Author

Peter C. Frommelt MD Adjunct Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Child
Heart Ventricles
Humans
Hypoplastic Left Heart Syndrome
Infant
Norwood Procedures
Pulmonary Artery
Stroke Volume
Treatment Outcome
Ventricular Function, Right