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.
Key Words
congenital
follow-up studies
Fontan procedure
heart defects
Abbreviations and Acronyms
BCPS
bidirectional cavopulmonary shunt
CI
confidence interval
HLHS
hypoplastic left heart syndrome
HR
hazards ratio
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Dr. d'Udekem is a Career Development Fellow of the National Heart Foundation of Australia (CR 10M 5339). This research project was supported by the Victorian Government's Operational Infrastructure Support Program. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.