Impact of Initial Shunt Type on Echocardiographic Indices in Children After Single Right Ventricle Palliations

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.

Loss of Ventricular Function After Bidirectional Cavopulmonary Connection: Who Is at Risk?

“…Retrospective studies of patients with functional SV with long-term follow-up data reported other risk factors that negatively impact prognosis including arrhythmia, atrioventricular valve regurgitation, and prolonged pleural effusion drainage [ 6 , 34 ]. Two large consortium studies (Single Ventricle Reconstruction Trial from North America [ 4 , 5 , 35 ] and the Australian and New Zealand Stage III Registry [ 8 ]) followed patients with functional SV physiology over time. In these studies, atrioventricular valve regurgitation and heterotaxy were predictors of mortality [ 8 ].…”

“…In these studies, atrioventricular valve regurgitation and heterotaxy were predictors of mortality [ 8 ]. But source of pulmonary blood flow (modified Blalock-Taussig shunt versus RV to the pulmonary artery conduit (Sano shunt)) at stage I did not change long-term prognosis [ 4 , 5 , 35 ].…”

Longitudinal Follow-Up of Children With HLHS and Association Between Norwood Shunt Type and Long-Term Outcomes: The SVR III Study

“…These findings are consistent with the findings of Sobh et al, 16 who found that RVEF by CMR was 54% among 6- to 11-year-old children with HLHS and Fontan circulation, and with previous findings from the SVR cohort showing no difference in RVEF between shunt groups as measured by echocardiography at 6 years of age. 17 Whereas systolic function is relatively preserved, further evaluation of the CMR and echocardiography data will be important to understand the effect of age and of shunt type on diastolic function and other indicators of cardiac dysfunction. The preteen period may be too early to detect systolic dysfunction by CMR.…”

“…Right ventricular function was measured with CMR as well as with 2-dimensional transthoracic echocardiography. Participants who underwent CMR were 10.9 years of age (interquartile range, 10.4-11.5) at the time of CMR, and, compared with those who did not have CMR, they had a significantly shorter hospital length of stay after the Norwood procedure (22 [interquartile range, [16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33] versus 28 days [interquartile range, 20-39]; P=0.02) and were more likely to be male (66% versus 47%; P=0.01; Table S2). Among the 177 participants who underwent CMR, RVEF was measurable in 168 (95%); the mean±SD for the overall cohort was 51.5±8.6% (RVPAS 51±9.…”

Hypoplastic Left Heart Syndrome Across the Lifespan: Clinical Considerations for Care of the Fetus, Child, and Adult

“…108 Serial monitoring of RV function with the use of echocardiography measures such as fractional area change, RV strain, and tricuspid annular plane systolic excursion can help in early detection and risk stratification. 107,[109][110][111][112][113] Although the initial selection of surgical strategy does not seem to have important impact on RV systolic function, 105,[114][115][116][117][118][119] strategies for medical preservation of the RV may be a promising avenue of future study. Recent data suggest that digoxin use in the interstage is associated with better survival, likely related to an effect of RV remodelling, 120 but other potential medical therapies deserve similar exploration.…”