Faculty Collaboration Database

Longitudinal Follow-Up of Children With HLHS and Association Between Norwood Shunt Type and Long-Term Outcomes: The SVR III Study. Circulation 2023 Oct 24;148(17):1330-1339

Date

10/05/2023

Pubmed ID

37795623

Pubmed Central ID

PMC10589429

DOI

10.1161/CIRCULATIONAHA.123.065192

Scopus ID

2-s2.0-85175256663 (requires institutional sign-in at Scopus site)   2 Citations

Abstract

OBJECTIVE: In the SVR trial (Single Ventricle Reconstruction), newborns with hypoplastic left heart syndrome were randomly assigned to receive a modified Blalock-Taussig-Thomas shunt (mBTTS) or a right ventricle-to-pulmonary artery shunt (RVPAS) at Norwood operation. Transplant-free survival was superior in the RVPAS group at 1 year, but no longer differed by treatment group at 6 years; both treatment groups had accumulated important morbidities. In the third follow-up of this cohort (SVRIII [Long-Term Outcomes of Children With Hypoplastic Left Heart Syndrome and the Impact of Norwood Shunt Type]), we measured longitudinal outcomes and their risk factors through 12 years of age.

METHODS: Annual medical history was collected through record review and telephone interviews. Cardiac magnetic resonance imaging (CMR), echocardiogram, and cycle ergometry cardiopulmonary exercise tests were performed at 10 through 14 years of age among participants with Fontan physiology. Differences in transplant-free survival and complication rates (eg, arrhythmias or protein-losing enteropathy) were identified through 12 years of age. The primary study outcome was right ventricular ejection fraction (RVEF) by CMR, and primary analyses were according to shunt type received. Multivariable linear and Cox regression models were created for RVEF by CMR and post-Fontan transplant-free survival.

RESULTS: Among 549 participants enrolled in SVR, 237 of 313 (76%; 60.7% male) transplant-free survivors (mBTTS, 105 of 147; RVPAS, 129 of 161; both, 3 of 5) participated in SVRIII. RVEF by CMR was similar in the shunt groups (RVPAS, 51±9.6 [n=90], and mBTTS, 52±7.4 [n=75]; P=0.43). The RVPAS and mBTTS groups did not differ in transplant-free survival by 12 years of age (163 of 277 [59%] versus 144 of 267 [54%], respectively; P=0.11), percentage predicted peak Vo₂ for age and sex (74±18% [n=91] versus 72±18% [n=84]; P=0.71), or percentage predicted work rate for size and sex (65±20% versus 64±19%; P=0.65). The RVPAS versus mBTTS group had a higher cumulative incidence of protein-losing enteropathy (5% versus 2%; P=0.04) and of catheter interventions (14 versus 10 per 100 patient-years; P=0.01), but had similar rates of other complications.

CONCLUSIONS: By 12 years after the Norwood operation, shunt type has minimal association with RVEF, peak Vo₂, complication rates, and transplant-free survival. RVEF is preserved among the subgroup of survivors who underwent CMR assessment. Low transplant-free survival, poor exercise performance, and accruing morbidities highlight the need for innovative strategies to improve long-term outcomes in patients with hypoplastic left heart syndrome.

REGISTRATION: URL: https://www.

CLINICALTRIALS: gov; Unique identifier: NCT0245531.

Author List

Goldberg CS, Trachtenberg F, William Gaynor J, Mahle WT, Ravishankar C, Schwartz SM, Cnota JF, Ohye RG, Gongwer R, Taylor M, Paridon S, Frommelt PC, Afton K, Atz AM, Burns KM, Detterich JA, Hill KD, Cabrera AG, Lewis AB, Pizarro C, Shah A, Sharma B, 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
Female
Follow-Up Studies
Heart Ventricles
Humans
Hypoplastic Left Heart Syndrome
Infant
Infant, Newborn
Male
Norwood Procedures
Protein-Losing Enteropathies
Pulmonary Artery
Stroke Volume
Treatment Outcome
Ventricular Function, Right