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

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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.

Introduction

Sudden cardiac death is a leading cause of mortality in adults with congenital heart disease (CHD) such that defibrillators are increasingly implanted in this growing population.1 Although most patients seem to adjust well to this therapy, considerable psychosocial issues have been reported in the general population with an implantable cardioverter-defibrillator (ICD).2 Nevertheless, there is a paucity of data that specifically address the impact of ICDs on quality of life and other patient-reported outcomes (PROs) in adults with CHD. Understanding the influence of ICDs on PROs is of particular importance considering the high baseline prevalence of psychosocial concerns in adults with CHD, including generalized anxiety, depression, and difficulties coping with a lifelong medical condition.3 Indeed, targeted therapy to reduce psychological distress has been recognized as an important component of comprehensive care for adults with CHD.4

Therefore, we sought to assess the impact of ICDs in the largest study of PROs in adults with CHD, the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease–International Study (APPROACH-IS), which enrolled >4000 patients from 15 countries across 5 continents.5 Outcomes of interest included quality of life, perceived health status, psychological distress, sense of coherence, illness perception, and health behaviors.

Section snippets

Patient selection

Design and methods of APPROACH-IS have previously been described (ClinicalTrials.gov Identifier NCT02150603).5 In brief, the study included adults (age ≥18 years) diagnosed with CHD before age 10 years with the physical, cognitive, and language capabilities required to complete self-reported questionnaires. A total of 4028 patients from 24 centers were recruited from the following countries via the International Society for Adult Congenital Heart Disease (ISACHD): Argentina, Australia, Belgium,

Study population

A total of 3188 patients met inclusion criteria for the APPROACH-IS ICD study: 107 with ICDs and 3081 weight-matched controls without ICDs. Patients with ICDs were distributed according to the following countries of domicile: Argentina 3, Australia 2, Belgium 10, Canada 16, Italy 1, Japan 3, Norway 12, Sweden 1, Switzerland 6, Taiwan 3, the Netherlands 10, and the United States 40. Characteristics of the 2 matched groups are summarized in Table 2. No statistically significant difference was

Discussion

To our knowledge, this is the largest study to assess the impact of ICDs on PROs in adults with CHD. Main findings include the following: (1) perceived health status, psychological distress, sense of coherence, and health behaviors are comparable in patients with and without ICDs, and in those with primary vs secondary prevention indications; (2) ICD recipients perceive their illness as more threatening than matched controls without ICDs; (3) the quality-of-life score (LAS) is significantly

Conclusion

In a large international study of PROs in adults with CHD, perceived health status, psychological distress, sense of coherence, and health behaviors were comparable in those with and without ICDs. However, ICDs were associated with a more threatening perception of illness. Patients with ICDs implanted for secondary prevention reported a worse quality-of-life score than their counterparts with primary prevention indications despite adjusting for complexity of CHD, heart failure, and other

References (25)

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    However, in patients with CHD, older age, severity of illness, and more advanced functional class symptoms predict worse PRO scores.31–33 Moreover, the presence of an implantable cardioverter-defibrillator has been associated with a more threatening view of illness in adults with CHD, with a worse quality of life in those with secondary prevention indications.34 The current study adds to the growing knowledge base by demonstrating that atrial arrhythmias should be considered among the important predictors of worse PROs, with an influence that extends across a broad spectrum of domains.

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This work was supported by the Research Fund–KU Leuven (Leuven, Belgium) Grant OT/11/033; Swedish Heart-Lung Foundation Grant 20130607; University of Gothenburg Centre for Person-centred Care; and Cardiac Children's Foundation (Taiwan) Grant CCF2013_02.

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