Heart failure admissions in adults with congenital heart disease; risk factors and prognosis
Introduction
An increasing number of patients with congenital heart disease (CHD) reach adulthood due to improved developments in paediatric cardiology, cardiac surgery and thorough follow-up. With these improvements survival has increased tremendously. However, adults with CHD are often faced with symptoms, sequelae, and complications from residual defects and interventions, including arrhythmias, endocarditis, and congestive heart failure [1], [2], [3], [4].
Several studies have investigated the occurrence and risk factors of arrhythmias and endocarditis in adults with CHD [5], [6], [7], [8]. Yet, only a few studies have focused on the occurrence of heart failure (HF) in adults with CHD [9], [10], [11], and it is unknown how often patients need to be admitted. Moreover, the prognosis of HF in this patient group is poorly described, while HF is one of the main causes of death in these patients [12], [13], [14]. Numerous studies have confirmed the poor prognosis in patients who develop (symptomatic) HF secondary to acquired heart disease. Both morbidity and mortality (after hospitalization) are high [15], [16]. Whether the prognosis of HF in patients with CHD is as poor as in patients with acquired heart disease is still unclear.
Risk factors of mortality after HF have been identified from clinical trials and population-based studies for patients with acquired heart disease [16], [17]. However, risk factors of mortality in adult CHD patients with HF may be different. Knowledge of mortality risk factors can be used to generate predictive models that can aid clinicians' decision making, in particular by identifying patients who are at high or low risk of death. Patients at high risk of death might benefit from early interventions and increased medical surveillance. Therefore, the aims of the present study were 1) to assess the frequency of HF-admissions in adults with CHD, 2) to identify risk factors of first HF-admission using simple clinical parameters and 3) to identify risk factors of mortality in adult CHD patients first hospitalized for HF using information routinely available to clinicians at hospital presentation.
Section snippets
CONCOR registration
The CONgenital CORvitia (CONCOR) Dutch national registry database has been described in detail [18]. Briefly, CONCOR aims to facilitate research into the aetiology of CHD and on its outcome. From November 2001, patients with CHD aged 18 years or older have been recruited and included by three independent, permanently employed research nurses. Clinical data such as diagnosis, clinical events, and procedures — classified using the European Pediatric Cardiac Code Short List coding scheme [19] — as
Results
Of 10,808 patients available for analysis, 5268 (49%) were male and median age was 37.0 years (range 18 to 92) at end of follow-up. During a median follow-up period of 21 years, 274 patients (2.5%) were admitted for HF. Median age at first HF-admission was 46.7 years (range 19.2 to 90.9) and 51% were male. The cumulative observed risk of admission for HF in adulthood was 1.2% at the age of 40 years and 5.8% at the age of 60 years. The overall incidence of first HF-admission at adult age was 1.2 (95%
Discussion
This comprehensive study is the first to show estimates of prevalence and incidence of HF-admission in adults with CHD. Of nearly 11,000 adult CHD patients 2.5% were admitted for HF during a median follow-up of 21 years. The incidence of first HF-admission was 1.2 per 1000 patient years, with increasing risk of HF-admission with increasing age. Main defect, multiple defects, surgery and pacemaker implantation in childhood were identified as risk factors of first HF-admission in adulthood.
Conclusions
The incidence of HF-admission in adults with CHD is 1.2 per 1000 patient-years, which is much higher than in the general population. Moreover, it is to be expected that the proportion of CHD patients admitted for HF will increase in this aging population. Mortality risk is substantially increased after HF-admission. This all emphasises the importance to identify patients at high risk of HF-admission and mortality after HF-admission, who might benefit from closer follow-up and earlier medical or
Acknowledgements
We thank all the Dutch medical institutions and their study coordinators for participating in the CONCOR project. Furthermore, we thank Lia Engelfriet, Irene Harms and Sylvia van den Busken of the Interuniversity Cardiology Institute of the Netherlands for their dedicated support of the CONCOR project.
The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology.
References (42)
- et al.
Long-term prognosis of congenital heart defects: a systematic review
Int J Cardiol
(2008) - et al.
Incidence and risk distribution of heart failure in adolescents and adults with congenital heart disease after cardiac surgery
Am J Cardiol
(2006) - et al.
Circumstances of death in adult congenital heart disease
Int J Cardiol
(2012) - et al.
Mortality in adult congenital heart disease: are national registries reliable for cause of death?
Int J Cardiol
(2011) - et al.
Hospitalizations in patients with heart failure: who and why
J Am Coll Cardiol
(2009) - et al.
Predictors of mortality after discharge in patients hospitalized with heart failure: an analysis from the Organized Program to Initiate Lifesaving Treatment in Hospitalized Patients with Heart Failure (OPTIMIZE-HF)
Am Heart J
(2008) - et al.
Task force 1: the changing profile of congenital heart disease in adult life
J Am Coll Cardiol
(2001) - et al.
Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study
J Am Coll Cardiol
(2000) - et al.
Mean arterial pressure: therapeutic goals and pharmacologic support
Crit Care Clin
(2010) - et al.
Arrhythmia and survival in patients > 18 years of age after the mustard procedure for complete transposition of the great arteries
Am J Cardiol
(1999)
Heart failure in the adult patient with congenital heart disease
J Card Fail
Mode of death in advanced heart failure: the Comparison of Medical, Pacing, and Defibrillation Therapies in Heart Failure (COMPANION) trial
J Am Coll Cardiol
Development and validation of a prediction model with missing predictor data: a practical approach
J Clin Epidemiol
Social burden and lifestyle in adults with congenital heart disease
Am J Cardiol
Rationale and design of a trial on the effect of angiotensin II receptor blockers on the function of the systemic right ventricle
Am Heart J
The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. The Euro Heart Survey on adult congenital heart disease
Eur Heart J
Congenital heart disease in adults: residua, sequelae, and complications of cardiac defects repaired at an early age
Rev Esp Cardiol
Gender and outcome in adult congenital heart disease
Circulation
Atrial arrhythmias in adults with congenital heart disease
Circulation
Ventricular arrhythmias and sudden death in adults after a Mustard operation for transposition of the great arteries
Eur Heart J
Thirty-year incidence of infective endocarditis after surgery for congenital heart defect
JAMA
Cited by (162)
Heart Failure Staging and Indications for Advanced Therapies in Adults with Congenital Heart Disease
2024, Heart Failure ClinicsEpidemiology and Definition of Heart Failure in Adult Congenital Heart Disease
2024, Heart Failure ClinicsEditorial commentary: Heart failure incidence and etiologies at young adult age
2024, Trends in Cardiovascular MedicineTrends in prevalence of major etiologies leading to heart failure in young patients: An integrative review
2024, Trends in Cardiovascular MedicineCitation Excerpt :Worldwide, 1.35–1.5 million children are born with CHDs each year [17]. The cumulative observed risk of admission for HF in adult patients with CHD was 1.2% at age 40 years [18]. Indeed, Sweden and Danish studies reported that 5.9% and 5.2% of young patients (≤ 50 yo) hospitalized for HF added previous CHD, respectively [3,13].
Prognostic role of inferior vena collapsibility index in congenital heart disease: A validation study
2024, International Journal of Cardiology