Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre.
Circulation. 2015 Sep 14;
Authors: Diller GP, Kempny A, Alonso-Gonzalez R, Swan L, Uebing A, Li W, Babu-Narayan S, Wort SJ, Dimopoulos K, Gatzoulis MA
Abstract
BACKGROUND: -Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centres has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary centre.
METHODS AND RESULTS: -We included 6,969 adult patients (age 29.9±15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age and gender matched population and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (IQR 5.2-14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%) and haemorrhage (5%), while perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, while patients with Eisenmenger syndrome, complex congenital heart disease and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient's age, whereas the proportion of patients dying from non-cardiac causes, such as cancer, increased.
CONCLUSIONS: -ACHD patients continue to be afflicted by increased mortality compared to the general population as they grow older. Highest mortality rates were observed amongst patients with complex ACHD, Fontan physiology and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of non-cardiac causes of death in contemporary ACHD patients.
PMID: 26369353 [PubMed - as supplied by publisher]