Predictors of survival after single-ventricle palliation: The impact of right ventricular dominance

Yves d'Udekem, Mary Xu, John Galati, Siming Lu, Ajay Iyengar, Igor Konstantinov, Gavin Wheaton, James Ramsay, Leeanne Grigg, Johnny Millar, Michael Cheung, Christian Brizard

    Research output: Contribution to journalArticlepeer-review

    132 Citations (Scopus)


    Objectives: This study examined survival after surgical palliation in children with single-ventricle physiology. Background: Contemporary surgical outcomes for the entire population of newborns undergoing single-ventricle palliation are unclear. Methods: In a single-center review of 499 consecutive patients undergoing univentricular palliation from 1990 to 2008, predictors of mortality were determined using multivariate risk analysis, stratified for each post-operative stay and interim states. Results: After 2000, the population comprised more patients with dominant right ventricle (66% vs. 36%) and hypoplastic left heart syndrome (HLHS) (47% vs. 13%). Median age at bidirectional cavopulmonary shunt (BCPS) decreased from 15 months (10 to 22 months) before 2000 to 4 months (3.3 to 9 months) thereafter. Survival rates at 1, 5, and 10 years were, respectively, 82% (95% confidence interval [CI]: 79% to 85%), 74% (95% CI: 70% to 78%), and 71% (95% CI: 67% to 75%). Throughout the study, atrioventricular valve regurgitation (hazard ratio [HR]: 1.8; p = 0.008), not having transposition (HR: 2.0; p = 0.013), and heterotaxia (HR: 2.0; p = 0.026) were predictors of mortality. The most potent risk factor was right ventricular (RV) dominance (HR: 2.2; p = 0.001) because of its impact before BCPS. HR for death in patients with RV dominance went from 2.8 (95% CI: 1.4 to 5.7; p = 0.005) before BCPS to 1.0 (95% CI: 0.5 to 2.1; p = 0.98) thereafter. Survival of patients with RV dominance, adjusted for the risk factors noted here, improved over the study period (p = 0.001). Conclusions: Considerable mortality is still observed during the first years of life among patients with single ventricle. RV dominance is the most important risk factor for death but only before BCPS.

    Original languageEnglish
    Pages (from-to)1178-1185
    Number of pages8
    JournalJournal of The American College of Cardiology
    Issue number13
    Publication statusPublished - 27 Mar 2012


    • congenital
    • follow-up studies
    • Fontan procedure
    • heart defects


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