TY - JOUR
T1 - Predictors of survival after single-ventricle palliation: The impact of right ventricular dominance
AU - d'Udekem, Yves
AU - Xu, Mary
AU - Galati, John
AU - Lu, Siming
AU - Iyengar, Ajay
AU - Konstantinov, Igor
AU - Wheaton, Gavin
AU - Ramsay, James
AU - Grigg, Leeanne
AU - Millar, Johnny
AU - Cheung, Michael
AU - Brizard, Christian
PY - 2012/3/27
Y1 - 2012/3/27
N2 - 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.
AB - 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.
KW - congenital
KW - follow-up studies
KW - Fontan procedure
KW - heart defects
UR - http://www.scopus.com/inward/record.url?scp=84858673072&partnerID=8YFLogxK
U2 - 10.1016/j.jacc.2011.11.049
DO - 10.1016/j.jacc.2011.11.049
M3 - Article
SN - 0735-1097
VL - 59
SP - 1178
EP - 1185
JO - Journal of The American College of Cardiology
JF - Journal of The American College of Cardiology
IS - 13
ER -