Circumstances of death in adult congenital heart disease

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Abstract

Background

Circumstances of death have been described for various cardiovascular diseases, but this study is the first for adults with congenital heart disease (CHD).

Methods

Review of medical records and additional information from treating cardiologists and general practitioners, for circumstances of all deaths in a national registry of over 8000 adults with CHD.

Results

Of 8595 patients, 231 (2.7%) patients died over 26,500 patient years. Main causes of death were progressive heart failure (26%) and sudden cardiac death (22%). Mortality was highest in the northern, most rural region of the country (p  0.05). Overall, death occurred out-of-hospital in approximately 35%, but more frequently in rural than in urban areas (55% versus 32%, p  0.05). Mortality was almost equally distributed throughout the seasons, although fall showed a slightly higher mortality rate. Cardiovascular death occurred suddenly in nearly 40%. Sudden cardiovascular death occurred in 8% during exercise, and most often out-of-hospital (62%). Of non-sudden cardiovascular deaths 18% had occurred out-of-hospital.

Conclusion

In adult patients with congenital heart disease, mortality shows substantial regional and subtle seasonal variation. Death usually occurs at rest; approximately 1 of 10 sudden cardiovascular deaths occur during exercise.

Introduction

An increasing number of patients with congenital heart disease (CHD) reach adulthood due to improved developments in paediatric cardiology, cardiac surgery and thorough monitoring [1]. However, high morbidity and premature death in adulthood have often been described [2], [3], [4], [5], [6]. Yet, little is known about the circumstances and modes of death in these patients.

Several studies have described regional, weekly and/or seasonal variations in cardiovascular mortality, especially in heart failure death, sudden cardiac death (SCD), death due to acute myocardial infarction and death from stroke [7], [8], [9], [10], [11], [12], [13], [14], [15], [16]. Whether such variation is also seen in adult patients with CHD has not been investigated. As CHD patients are at increased risk for premature death, there is a need for more detailed research on modes and circumstances of death, as better insight may help identify and avoid high-risk situations, and improve survival.

Although we know that some deaths occur in high-risk situations (e.g. perioperative [17]), others are unexpected events [18] occurring in various settings. Heavy physical exercise as trigger for sudden death (SD) in adults with CHD has been suggested, but is not as well reported as for coronary artery disease, (isolated) coronary artery anomalies, and hypertrophic cardiomyopathy [19], [20], [21], [22], [23].

We systematically reviewed all deaths recorded in the CONCOR registry, a national database for adults with CHD, to assess the circumstances of death.

Section snippets

CONCOR registry

The CONCOR (CONgenital CORvitia) Dutch national registry and DNA bank of adult patients with congenital heart disease database has been described in detail previously [24]. In brief, CONCOR aims to facilitate research into the aetiology of CHD and on its outcome. Between November 2001 and January 2010, nearly 12,000 patients with CHD aged 18 years or older have been included through their treating cardiologist or via response to advertisements in local and national media. Clinical data such as

Results

Of 8595 patients, 231 (2.7%) died during a total follow up period of 26,500 patient years (mean follow up period was 37.1 ± 0.2 months). Median age at death was 48.4 years (range, 20.2 to 91.2 years); 57% were males. Table 1 shows the distribution of defects in CONCOR and among the deceased patients. The most common defects in CONCOR were atrial septal defect (17%), ventricular septal defect (16%), aortic stenosis/bicuspid aortic valve (14%), tetralogy of Fallot (10%), and aortic coarctation (10%).

Discussion

This is the first comprehensive study assessing circumstances of death in adult patients with CHD. We found regional mortality variation and a peak in fall. Death usually occurred at rest, with merely 8% of sudden cardiovascular death occurring during exercise. A quarter of non-sudden cardiovascular deaths was preceded by signs of infection.

Our results show that progressive heart failure and sudden cardiac death are the most common causes of death in adult patients with CHD, as has been

Acknowledgments

The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology [46].

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