• A Carla Zomer, Ilonca Vaartjes, Cuno S P M Uiterwaal, Enno T van der Velde, Lambert F M van den Merkhof, Leo H B Baur, Tieneke J M Ansink, Luc Cozijnsen, Petronella G Pieper, Folkert J Meijboom, Diederick E Grobbee, and Barbara J M Mulder.
• Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
• Int. J. Cardiol. 2012 Jan 26;154(2):168-72.

BackgroundCircumstances of death have been described for various cardiovascular diseases, but this study is the first for adults with congenital heart disease (CHD).MethodsReview 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.ResultsOf 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.ConclusionIn 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.Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

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