Key result
Greater than mild tricuspid regurgitation increased from 12.1% to 24.3% post-Norwood (p<0.001) and was associated with decreased transplant-free survival (HR 1.77 pre-Norwood) through 14 months in infants with single right ventricle anomalies; TV intervention at Norwood or Stage II was not associated with improved survival and had higher death/transplant rates.
Why the study?
Tricuspid regurgitation is common in single right ventricle anomalies and affects outcomes during staged surgical palliations, but its longitudinal course and the impact of tricuspid valve intervention required evaluation.
Does tricuspid valve intervention or the presence of > mild tricuspid regurgitation impact transplant-free survival in children with single right ventricle anomalies undergoing staged surgical palliation?
RCT (n=543)
Open-label
randomized to shunt type (modified Blalock-Taussig or right ventricle-to-pulmonary artery)
Yes
Does tricuspid valve intervention or the presence of > mild tricuspid regurgitation impact transplant-free survival in children with single right ventricle anomalies undergoing staged surgical palliation?
Answers are grounded in this paper and cite their sources.
In children with single right ventricle anomalies, > mild tricuspid regurgitation and early tricuspid valve intervention are associated with worse transplant-free survival, highlighting the need for improved valve management strategies.
Tricuspid regurgitation (TR) is common in single right ventricle (RV) anomalies and affects outcomes during staged surgical palliations. The Pediatric Heart Network’s (PHN) Single Ventricle Reconstruction trial followed children with single RV to age 6 years. This study compared serial echocardiographic measures of TR during staged palliations and determine the impact of TR and tricuspid valve (TV) intervention on transplant-free survival in the PHN cohort. Study echocardiograms were performed at 6 pre-determined times: baseline, post-Norwood, pre-stage II, age 14 months, pre-Fontan, and age 6 years. TR was categorized as ≤ mild or > mild. McNemar’s test compared TR across time; t-tests compared RV size/function by TR category; and survival analysis assessed the effect of > mild TR on transplant-free survival. TR was assessed in 543 participants prior to Norwood. From baseline to post-Norwood, those with > mild TR increased from 12 to 24% ( p < 0.001). Thereafter, the proportion of surviving participants with > mild TR was stable. Participants with > mild TR had increased baseline TV annular area, and increased RV volume but not decreased function. Transplant-free survival at 6 years was lower with > mild TR at any time except pre-Fontan. 78 TV interventions occurred in 66 participants. Those with TV intervention at the Norwood and/or Stage II had a higher rate of death/transplant than those without or those who had a later intervention. At baseline, post Norwood and pre-stage II, > mild TR is associated with decreased transplant-free survival. TV intervention at the Norwood or Stage II operations was not associated with improved survival.
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Cnota et al. (2026) conducted an RCT in Infants with single right ventricle anomalies undergoing Norwood procedure (n=543). Tricuspid valve (TV) intervention during Norwood or Stage II surgery vs. No TV intervention was evaluated on Transplant-free survival to 6 years (HR 1.77 for > mild TR pre-Norwood (95% CI 1.23–2.56); HR 1.5 for > mild TR (95% CI 1.1–2.1); HR 2.7 for TV intervention (95% CI 1.6–4.6), p=p < 0.001 increase in > mild TR post-Norwood; p < 0.05 for survival differences at multiple time points except pre-Fontan). Greater than mild tricuspid regurgitation increased from 12.1% to 24.3% post-Norwood (p<0.001) and was associated with decreased transplant-free survival (HR 1.77 pre-Norwood) through 14 months in infants with single right ventricle anomalies; TV intervention at Norwood or Stage II was not associated with improved survival and had higher death/transplant rates.
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