Long-term Outcomes of Children With Hypoplastic Left Heart Syndrome and the Impact of Norwood Shunt Type

NCT ID: NCT02455531

Last Updated: 2025-11-10

Basic Information

• Recruitment Status: ACTIVE_NOT_RECRUITING
• Total Enrollment: 237 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2015-06-30
• Study Completion Date: 2025-12-31

Brief Summary

The purpose of this study is to compare direct and indirect measures of right ventricular (RV) systolic and diastolic function between 11 year old subjects who had been randomly assigned to receive a right ventricle to pulmonary artery shunt (RVPAS) vs. a modified Blalock-Taussig shunt (MBTS) at the time of the Norwood operation.

Detailed Description

The Single Ventricle Reconstruction (SVR) trial was the first multicenter, randomized clinical trial to compare two operations in the field of congenital heart disease.8,11 Children with HLHS and other related single RV lesions were enrolled and randomized to receive either a MBTS or a RVPAS at the time of the initial Norwood procedure. This landmark study provided extraordinary insight not only into the consequences of both shunt types, but also into the course, treatment responses and short- and mid-term outcomes for these medically complex patients. Through the SVR Trial and SVR Extension Study (SVR II), outcomes, including but not limited to the primary outcome of transplant-free survival, have now been evaluated in this patient cohort when the last enrolled patient reached 12 months and again at 3 years of age. While early post-operative transplant-free survival during the interstage period7 and at one year8 was better for those children randomized to a RVPAS, survival by the 3-year evaluation appeared equivalent between the two shunt types. Moreover, RVEF was somewhat diminished and the number of interventions was higher in the RVPAS group.12 These findings raised concern that the RV dysfunction in the RVPAS group may be progressive, leading to significantly worse long-term outcomes; if so, the benefits of the RVPAS for short-term survival may be outweighed by longer-term morbidity and mortality. Thus, the optimal surgical approach for newborns with HLHS and related single RV lesions remains unclear.

The Pediatric Heart Network (PHN) Investigators have a unique opportunity and responsibility to analyze the effect of the type of systemic-to-pulmonary artery shunt placed during the Norwood procedure on longer-term survival, as well as to define its effect on other long-term outcomes in this multi-institutional cohort of exquisitely characterized subjects with single RV lesions. As subjects enrolled in the SVR cohort approach a decade of age, the investigators aim: 1) to determine if shunt type at the time of Norwood operation is associated with any long-term differences in cardiac function, survival, or contributors to quality of life; and 2) to characterize long-term outcomes and determine risk factors other than shunt type for adverse long-term outcomes in children with HLHS and other related single ventricle anomalies.

Conditions

Heart Defects

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Study Groups

Transplant-free survivors

Transplant-free survivors of the SVR cohort (All SVR survivors are eligible to be followed for vital status.)

No interventions assigned to this group

Eligibility Criteria

Inclusion Criteria

• All SVR study cohort members will be contacted to assess for vital status. Transplant free survivors will be approached to participate in the in-person assessment.

Exclusion Criteria

• Patients who have undergone cardiac transplantation or biventricular conversion from all outcomes other than vital status.
• Those with pacemakers will be excluded from the CMR, and patients <130 cm in height will be excluded from the exercise test.

• Minimum Eligible Age: 10 Years
• Maximum Eligible Age: 12 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

National Heart, Lung, and Blood Institute (NHLBI)

NIH

Sponsor Role: collaborator

Carelon Research

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

References

Miller TA, Sharma B, Gongwer R, Trachtenberg FL, Newburger JW, Goldberg CS, Gustafson KE, Gaynor JW, Votava-Smith JK, Lambert LM, Sananes R, Kral MC, Tsang R, Heinrich KP, Cnota J, Shah A, Ilardi D; Pediatric Heart Network Investigators. Neurodevelopmental Outcomes in Early Adolescence: The Pediatric Heart Network's Single Ventricle Reconstruction Trial. Circulation. 2025 Oct 28;152(17):1246-1261. doi: 10.1161/CIRCULATIONAHA.125.074523. Epub 2025 Jul 17.

Reference Type: DERIVED

PMID: 40671650 (View on PubMed)

Provided Documents

Document Type: Study Protocol and Statistical Analysis Plan

View Document

Document Type: Informed Consent Form

View Document

Other Identifiers

U10HL068270

Identifier Type: NIH

Identifier Source: secondary_id

View Link

U10HL068270-4b

Identifier Type: -

Identifier Source: org_study_id