Major morbidity rife in single ventricle patients

Death or major morbidity is prevalent in patients with single ventricles, reveals a study.

Researchers assessed global morbidity and longitudinal outcomes in the Single Ventricle Reconstruction (SVR) trial using a novel hierarchically ranked composite endpoint, which included death, transplant, parent-reported adaptive function and quality of life, right ventricular function, major complications, and hospital stay.

Some 549 participants were included and subsequently ranked by their worst outcomes over 16 years of follow-up. The research team compared outcomes by shunt type using risk-adjusted ordinal logistic regression, with Kaplan-Meier, Cox regression, and win ratio analyses for sensitivity.

Of the SVR trial participants, 480 (87 percent) developed major morbidity or died.

In multivariable analysis, shunt type showed an association with pre-Norwood tricuspid regurgitation, with no shunt-related difference in outcomes in those with no or mild pre-Norwood tricuspid regurgitation (odds ratio [OR], 1.3, 95 percent confidence interval [CI], 0.9‒1.8; p=0.14) but worse outcomes in right ventricle-to-pulmonary artery (RVPAS) shunt participants with moderate or severe pre-Norwood tricuspid regurgitation (OR, 0.4, 95 percent CI, 0.2‒0.9; p=0.03).

These findings were confirmed in sensitivity analyses, with increased mortality in RVPAS participants with moderate or severe pre-Norwood tricuspid regurgitation (hazard ratio, 5.4, 95 percent CI, 2.2‒13.1; p=0.0002) in Cox regression.

Notably, prematurity and site independently predicted worse outcomes.

“Although overall outcomes did not differ by shunt type, the observed interaction between shunt type and severity of tricuspid regurgitation highlights an important area for future investigation,” the investigators said.