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Impact of Initial Shunt Type on Echocardiographic Indices in Children After Single Right Ventricle Palliations
Peter C Frommelt1,2, Chenwei Hu3, Felicia Trachtenberg3
1Department of Pediatrics, Division of Pediatric Cardiology, Children's Hospital of Wisconsin, Milwaukee (P.C.F., J.S.).
Insights
Children with single right ventricle (RV) anomalies show similar heart function regardless of initial shunt type after the Norwood procedure. However, poor RV systolic function early on predicts worse long-term survival.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Palliation
Background:
- Single right ventricle (RV) anomalies present complex challenges in pediatric cardiology.
- The choice of shunt during the Norwood procedure may influence long-term RV size and function.
- Understanding these early differences is crucial for optimizing surgical palliation strategies.
Purpose of the Study:
- To compare echocardiographic indices of RV, neoaortic, and tricuspid valve size and function between different shunt types after the Norwood procedure.
- To identify potential long-term impacts of shunt strategy on heart remodeling in children with single RV.
- To assess the predictive value of early RV function on late outcomes.
Main Methods:
- A randomized trial comparing modified Blalock-Taussig shunt versus RV-to-pulmonary-artery shunt in 241 subjects undergoing the Norwood procedure.
- Echocardiographic assessments of RV, neoaortic, and tricuspid valve size and function were performed at 14 months, pre-Fontan, and 6 years.
- Statistical analysis to identify differences in cardiac indices and evaluate predictors of late mortality/transplant.
Main Results:
- At 6 years, no significant differences in RV size and function were observed between shunt groups, except for a larger indexed neoaortic area in the modified Blalock-Taussig shunt group.
- RV ejection fraction improved from pre-Fontan to 6 years in the RV-to-pulmonary artery shunt group, but remained stable in the modified Blalock-Taussig shunt group.
- Diminished RV ejection fraction (<40%) at 14 months was a significant predictor of late death or transplant (HR, 3.18).
Conclusions:
- Shunt type in the Norwood procedure does not significantly impact RV size and function by 6 years post-staged palliation.
- Beneficial remodeling of the RV and valves occurs by 6 years, irrespective of the initial shunt.
- Early RV systolic function is a critical determinant of long-term survival in single RV patients, independent of shunt strategy.
Abstract:
Background Heart size and function in children with single right ventricle (RV) anomalies may be influenced by shunt type at the Norwood procedure. We sought to identify shunt-related differences during early childhood after staged surgical palliations using echocardiography. Methods We compared echocardiographic indices of RV, neoaortic, and tricuspid valve size and function at 14 months, pre-Fontan, and 6 years in 241 subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt or RV-to-pulmonary-artery shunt. Results At 6 years, the shunt groups did not differ significantly in any measure except for increased indexed neoaortic area in the modified Blalock-Taussig shunt. RV ejection fraction improved between pre-Fontan and 6 years in the RV-to-pulmonary artery shunt group but was stable in the modified Blalock-Taussig shunt group. For the entire cohort, RV diastolic and systolic size and functional indices were improved at 6 years compared with earlier measurements, and indexed tricuspid and neoaortic annular area decreased from 14 months to 6 years. The prevalence of ≥moderate tricuspid and neoaortic regurgitation was uncommon and did not vary by group or time period. Diminished RV ejection fraction at the 14-month study was predictive of late death/transplant; the hazard of late death/transplant when RV ejection fraction was <40% was tripled (hazard ratio, 3.18; 95% CI, 1.41-7.17). Conclusions By 6 years after staged palliation, shunt type has not impacted RV size and function, and RV and valvar size and function show beneficial remodeling. Poor RV systolic function at 14 months predicts worse late survival independent of the initial shunt type. Clinical Trial Registration URL: https://www.clinicaltrials.gov . Unique identifier: NCT00115934.
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