Objective: Patients with single-ventricle heart disease experience early and late failure during and after staged palliation. Little is known about the factors related to continued risk of transplantation and mortality after completion of staged palliation. The long-term outcome of patients with single-ventricle disease who require a gastric fundoplication early in life has not been assessed.
Methods: A total of 155 patients with single-ventricle disease who survived their first-stage palliative procedure were enrolled in a research registry. Demographic and anatomic variables were collected, and the families were contacted every 6 months for prospective documentation of transplant-free survival. Medical records were reviewed for the details of noncardiac surgical procedures. Univariate and multivariate regression analyses were performed to determine the impact of early gastric surgery on late transplant-free survival.
Results: There were 93 male patients, median gestational age was 38 weeks, and birth weight was 3.2 kg. Sixty-five patients (42%) had hypoplastic left heart syndrome. Twelve patients (7.7%) had a genetic syndrome. Thirty-two patients (21%) had a fundoplication or a gastrostomy tube at less than 2 years of age. Median follow-up was 4.3 years (range, 79 days to 10 years). Race, gender, gestational age, birth weight, and genetic syndrome did not alter midterm transplant-free survival. Need for fundoplication or gastrostomy was an independent risk factor for decreased transplant-free survival (P = .003; hazard ratio, 4.29), which was unchanged when adjusted for all covariates.
Conclusions: The need for early fundoplication or gastrostomy is associated with decreased transplant-free survival for patients with palliated single-ventricle heart disease.
Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.