title: Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre.
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.
  
  
  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.affiliation: 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.).
authors.forename: Gerhard-Paul
authors.initials: GP
authors.lastname: Diller
authors.affiliation: 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.
authors.forename: Aleksander
authors.initials: A
authors.lastname: Kempny
authors.affiliation: 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.).
authors.forename: Rafael
authors.initials: R
authors.lastname: Alonso-Gonzalez
authors.affiliation: 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.).
authors.forename: Lorna
authors.initials: L
authors.lastname: Swan
authors.affiliation: 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.).
authors.forename: Anselm
authors.initials: A
authors.lastname: Uebing
authors.affiliation: 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.).
authors.forename: Wei
authors.initials: W
authors.lastname: Li
authors.affiliation: 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.).
authors.forename: Sonya
authors.initials: S
authors.lastname: Babu-Narayan
authors.affiliation: 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.).
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Wort
authors.affiliation: 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.).
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.affiliation: 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.).
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002423:Cause of Death / Q000639:trends; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000401:mortality*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D062606:Tertiary Care Centers / Q000639:trends*; D055815:Young Adult
keywords: heart defects, congenital; heart failure; mortality; sudden cardiac death; survival