Long-term survival in patients with univentricular heart: A nationwide, register-based cohort study

Abstract

Background

Children with univentricular heart (UVH) have a limited life expectancy without early treatment. Long-term survival in UVH, in an unselected nationwide cohort, is unclear.

Objectives

To determine long-term survival in patients with UVH including non-operated patients compared with a control population in Sweden.

Methods

Patients with UVH born between 1970 and 2017 were identified from the National Registers and were matched for birth year and sex with 10 individuals without congenital heart disease. Follow-up was from birth until death, transplantation, or the end of study. Mortality risk was estimated by Cox proportional regression models and Kaplan–Meier survival analysis.

Results

We included 5075 patients with UVH including 758 (14.9%) patients with hypoplastic left heart syndrome (HLHS), and 50,620 matched controls. Median follow-up time was 13.6 (IQR 0.7; 26.8) years. The hazard ratio for death in patients with UVH was 53.0 (95% confidence interval, 48.0–58.6), and for HLHS, 163.5 (95% CI, 124.3–215.2). In patients with HLHS, 84% of those who were born between 1982 and 1993 died or had transplantation during the first year of life compared with 29% born between 2006 and 2017. In patients with UVH without HLHS, death/transplantation in the first year of life declined from 36% in those born between 1970 and 1981 to 8.7% in those born between 2006 and 2017.

Conclusions

The risk of mortality was >50 times higher in patients with UVH than in controls. The survival rate increased with a later decade of birth but was still <75% in patients born with HLHS.