Heart failure admissions in adults with congenital heart disease; risk factors and prognosis

https://doi.org/10.1016/j.ijcard.2013.03.003Get rights and content

Abstract

Background

Heart failure (HF) is a serious complication and often the cause of death in adults with congenital heart disease (CHD). Therefore, our aims were to determine the frequency of HF-admissions, and to assess risk factors of first HF-admission and of mortality after first HF-admission in adults with CHD.

Methods

The Dutch CONCOR registry was linked to the Hospital Discharge Registry and National Mortality Registry to obtain data on HF-admissions and mortality. Risk factors for both HF-admission and mortality were assessed using Cox regression models.

Results

Of 10,808 adult patients (49% male), 274 (2.5%) were admitted for HF during a median follow-up period of 21 years. The incidence of first HF-admission was 1.2 per 1000 patient-years, but the incidence of HF itself will be higher. Main defect, multiple defects, and surgical interventions in childhood were identified as independent risk factors of HF-admission. Patients admitted for HF had a five-fold higher risk of mortality than patients not admitted (hazard ratio (HR) = 5.3; 95% confidence interval 4.2–6.9). One- and three-year mortality after first HF-admission were 24% and 35% respectively. Independent risk factors for three-year mortality after first HF-admission were male gender, pacemaker implantation, admission duration, non-cardiac medication use and high serum creatinine.

Conclusions

The incidence of HF-admission in adults with CHD is 1.2 per 1000 patient-years. Mortality risk is substantially increased after HF-admission, which emphasises the importance to identify patients at high risk of HF-admission. These patients might benefit from closer follow-up and earlier medical interventions. The presented risk factors may facilitate surveillance.

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.

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