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

Ayse-Gül Öztürk1,2, Mikael Dellborg3, Anna Damlin4,5

  • 1Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden.

International Journal of Cardiology. Congenital Heart Disease
|December 23, 2024
PubMed

Insights

Children with univentricular heart (UVH) face significantly higher mortality risks, over 50 times greater than controls. While survival has improved for UVH patients born more recently, those with hypoplastic left heart syndrome (HLHS) still have a less than 75% survival rate.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease Epidemiology
  • Public Health

Background:

  • Univentricular heart (UVH) conditions significantly limit life expectancy without timely intervention.
  • Long-term survival data for univentricular heart patients in a comprehensive, unselected national cohort remain largely undefined.
  • Understanding survival trends is crucial for improving patient outcomes and healthcare strategies.

Purpose of the Study:

  • To ascertain the long-term survival rates of patients diagnosed with univentricular heart (UVH).
  • To compare survival outcomes in UVH patients, including those not surgically treated, against a general population control group in Sweden.
  • To analyze survival trends over time based on decade of birth.

Main Methods:

  • Utilized Swedish National Registers to identify 5075 patients with UVH born between 1970 and 2017.
  • Matched each UVH patient with 10 control individuals of the same birth year and sex without congenital heart disease.
  • Employed Cox proportional regression models and Kaplan-Meier survival analysis for mortality risk estimation, with follow-up until death or transplantation.

Main Results:

  • The overall mortality risk for UVH patients was over 53 times higher than for controls (Hazard Ratio 53.0).
  • Patients with hypoplastic left heart syndrome (HLHS) faced an even greater mortality risk (Hazard Ratio 163.5).
  • First-year survival improved significantly for both UVH and HLHS patients born in later decades, though HLHS survival remained below 75%.

Conclusions:

  • Patients with UVH exhibit a substantially elevated mortality risk compared to the general population.
  • Survival rates for UVH patients have improved over successive birth decades, indicating progress in treatment and care.
  • Despite improvements, long-term survival for patients with HLHS remains a critical concern, necessitating continued research and therapeutic advancements.
Abstract

Keywords:
NationwideRegistry studySurvivalUniventricular heart

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