Heart Failure and Patient-Reported Outcomes in Adults With Congenital Heart Disease from 15 Countries

Abstract

Background Heart failure (HF) is the leading cause of mortality and associated with significant morbidity in adults with congenital heart disease. We sought to assess the association between HF and patient-report outcomes in adults with congenital heart disease. Methods and Results As part of the APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study), we collected data on HF status and patient-reported outcomes in 3959 patients from 15 countries across 5 continents. Patient-report outcomes were: perceived health status (12-item Short Form Health Survey), quality of life (Linear Analogue Scale and Satisfaction with Life Scale), sense of coherence-13, psychological distress (Hospital Anxiety and Depression Scale), and illness perception (Brief Illness Perception Questionnaire). In this sample, 137 (3.5%) had HF at the time of investigation, 298 (7.5%) had a history of HF, and 3524 (89.0%) had no current or past episode of HF. Patients with current or past HF were older and had a higher prevalence of complex congenital heart disease, arrhythmias, implantable cardioverter-defibrillators, other clinical comorbidities, and mood disorders than those who never had HF. Patients with HF had worse physical functioning, mental functioning, quality of life, satisfaction with life, sense of coherence, depressive symptoms, and illness perception scores. Magnitudes of differences were large for physical functioning and illness perception and moderate for mental functioning, quality of life, and depressive symptoms. Conclusions HF in adults with congenital heart disease is associated with poorer patient-reported outcomes, with large effect sizes for physical functioning and illness perception. Registration URL: https://clinicaltrials.gov; Unique identifier: NCT02150603.

Keywords: adult congenital heart disease; heart failure; patient‐reported outcomes; quality of life.

Figure 1. The occurrence of heart failure in different congenital heart disease severities. Patients are assigned to the types of heart defect in a mutually exclusive way.

When >1 defect was present in patients, the patient was assigned to the defect of highest complexity or with the biggest functional impact. ASD indicates atrial septal defect; CHD, congenital heart disease; CCTGA, congenitally corrected transposition of great arteries; HF, heart failure; PDA, patent ductus arteriosus; RVOTO, right ventricle outflow track obstruction; and VSD, ventricular septal defect.

Figure 2. Patient characteristics associated with past/current history of heart failure.

A, Comparison of current heart failure versus never heart failure (reference). B, Comparison of past heart failure versus never heart failure (reference). CHF indicates chronic heart failure; ICD, implantable cardioverter‐defibrillator; and OR, odds ratio.

Figure 3. Standardized effect size (Cohen’s d) for patients with congenital heart disease with current or past heart failure versus no heart failure.

Scores: mean (95% CI). HADS‐A indicates Hospital Anxiety and Depression Scale—Anxiety; HADS‐D, Hospital Anxiety and Depression Scale—Depression; HF, heart failure; IPQ, Illness Perception Questionnaire; LAS QOL, Linear Analog Scale Quality of Life; MCS, Mental Component Summary; PCS, Physical Component Summary; SOC, Sense of Coherence; and SWLS, Satisfaction with Life Scale.