Implantable cardioverter-defibrillators and patient-reported outcomes in adults with congenital heart disease: An international study

Heart Rhythm. 2020 May;17(5 Pt A):768-776. doi: 10.1016/j.hrthm.2019.11.026. Epub 2019 Nov 30.


Background: Implantable cardioverter-defibrillators (ICDs) are increasingly being used to prevent sudden death in the growing population of adults with congenital heart disease (CHD). However, little is known about their impact on patient-reported outcomes (PROs).

Objective: The purpose of this study was to assess and compare PROs in adults with CHD with and without ICDs.

Methods: A propensity-based matching weight analysis was conducted to evaluate PROs in an international cross-sectional study of adults with CHD from 15 countries across 5 continents.

Results: A total of 3188 patients were included: 107 with ICDs and 3081 weight-matched controls without ICDs. ICD recipients were an average age of 40.1 ± 12.4 years, and >95% had moderate or complex CHD. Defibrillators were implanted for primary and secondary prevention in 38.3% and 61.7%, respectively. Perceived health status, psychological distress, sense of coherence, and health behaviors did not differ significantly among patients with and without ICDs. However, ICD recipients had a more threatening view of their illness (relative % difference 8.56; P = .011). Those with secondary compared to primary prevention indications had a significantly lower quality-of-life score (Linear Analogue Scale 72.0 ± 23.1 vs 79.2 ± 13.0; P = .047). Marked geographic variations were observed. Overall sense of well-being, assessed by a summary score that combines various PROs, was significantly lower in ICD recipients (vs controls) from Switzerland, Argentina, Taiwan, and the United States.

Conclusion: In an international cohort of adults with CHD, ICDs were associated with a more threatening illness perception, with a lower quality of life in those with secondary compared to primary prevention indications. However, marked geographic variability in PROs was observed.

Keywords: Adult congenital heart disease; Implantable cardioverter-defibrillator; Patient-reported outcomes; Quality of life; Sudden cardiac death.

MeSH terms

  • Adult
  • Cross-Sectional Studies
  • Death, Sudden, Cardiac / epidemiology
  • Death, Sudden, Cardiac / etiology
  • Death, Sudden, Cardiac / prevention & control
  • Defibrillators, Implantable*
  • Heart Defects, Congenital* / complications
  • Heart Defects, Congenital* / epidemiology
  • Heart Defects, Congenital* / therapy
  • Humans
  • Middle Aged
  • Patient Reported Outcome Measures
  • Quality of Life
  • United States / epidemiology