Gerhard-Paul Diller1, Aleksander Kempny2, Rafael Alonso-Gonzalez1, Lorna Swan1, Anselm Uebing1, Wei Li1, Sonya Babu-Narayan1, Stephen J Wort1, Konstantinos Dimopoulos1, Michael A Gatzoulis1. 1. From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom (G.-P.D., A.K., R.A.-G., L.S., A.U., W.L., S.B.-N., S.J.W., K.D., M.A.G.); NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, United Kingdom (G.-P.D., A.K., R.A.-G., L.S., A.U., W.L., S.B.-N., S.J.W., K.D., M.A.G.); National Heart and Lung Institute, Imperial College School of Medicine, London, United Kingdom (G.-P.D., A.K., R.A.-G., L.S., A.U., W.L., S.B.-N., S.J.W., K.D., M.A.G.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiology and Angiology, University Hospital Muenster, Germany (G.-P.D.). 2. From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom (G.-P.D., A.K., R.A.-G., L.S., A.U., W.L., S.B.-N., S.J.W., K.D., M.A.G.); NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, United Kingdom (G.-P.D., A.K., R.A.-G., L.S., A.U., W.L., S.B.-N., S.J.W., K.D., M.A.G.); National Heart and Lung Institute, Imperial College School of Medicine, London, United Kingdom (G.-P.D., A.K., R.A.-G., L.S., A.U., W.L., S.B.-N., S.J.W., K.D., M.A.G.); and Division of Adult Congenital and Valvular Heart Disease, Department of Cardiology and Angiology, University Hospital Muenster, Germany (G.-P.D.). a.kempny@rbht.nhs.uk.
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 centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center. METHODS AND RESULTS: We included 6969 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 sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 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 hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas 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 noncardiac causes, such as cancer, increased. CONCLUSIONS: ACHD patients continue to be afflicted by increased mortality in comparison with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.
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 centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center. METHODS AND RESULTS: We included 6969 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 sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 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 hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas 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 noncardiac causes, such as cancer, increased. CONCLUSIONS: ACHD patients continue to be afflicted by increased mortality in comparison with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.
Authors: Jill M Steiner; James N Kirkpatrick; Susan R Heckbert; James Sibley; James A Fausto; Ruth A Engelberg; J Randall Curtis Journal: Congenit Heart Dis Date: 2018-09-19 Impact factor: 2.007
Authors: Alexander Van De Bruaene; Lukas Meier; Walter Droogne; Pieter De Meester; Els Troost; Marc Gewillig; Werner Budts Journal: Heart Fail Rev Date: 2018-01 Impact factor: 4.214