RESUMO
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.