title: Cause of death in adults with congenital heart disease - An analysis of the German National Register for Congenital Heart Defects.
abstract: BACKGROUND
  Due to the great advances in the care of patients with congenital heart disease (CHD), mortality has decreased significantly over the last decades. Nonetheless, mortality for adults with congenital heart disease (ACHD) is still higher than for the general population. An analysis regarding causes of death in a nationwide contemporary cohort of ACHD is lacking.
  
  
  METHODS
  A well-characterized cohort of the German National Register for Congenital Heart Defects was screened for patients over the age of 18 years who died between January 2001 and January 2015. Data relating to the cardiac diagnosis, symptoms, operations, interventions, comorbidities, and causes of death were analyzed.
  
  
  RESULTS
  During a median follow-up of 3.67 years (IQR 1.32-9.41), 239 (9.2%) out of 2596 patients died during the study period (110 female (46%), mean age at death 39.8 ± 17.8 years). The majority of these deaths was CHD-related (171 patients (71.5%)). Leading causes of death were heart failure (n=66, 27.6%), and sudden cardiac death (n=55, 23.0%). Deceased patients had a more complex CHD and more extracardiac comorbidities compared with living patients.
  
  
  CONCLUSIONS
  Causes of death of ACHD patients in a large contemporary cohort from a national register are in the majority still CHD-related, with heart failure being the leading cause of death. Additionally, extracardiac comorbidities gain increasing importance.
authors.affiliation: National Register for Congenital Heart Defects, Berlin, Germany; Department of Cardiology & Angiology, Hannover Medical School, Hannover, Germany; Competence Network for Congenital Heart Defects, Berlin, Germany.
authors.forename: Claudia C
authors.initials: CC
authors.lastname: Engelings
authors.affiliation: National Register for Congenital Heart Defects, Berlin, Germany; Competence Network for Congenital Heart Defects, Berlin, Germany.
authors.forename: Paul C
authors.initials: PC
authors.lastname: Helm
authors.affiliation: Competence Network for Congenital Heart Defects, Berlin, Germany; Department of Paediatric Cardiology, Saarland University Medical Center, Homburg, Germany.
authors.forename: Hashim
authors.initials: H
authors.lastname: Abdul-Khaliq
authors.affiliation: National Register for Congenital Heart Defects, Berlin, Germany; Competence Network for Congenital Heart Defects, Berlin, Germany; German Pediatric Heart Centre, Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany.
authors.forename: Boulos
authors.initials: B
authors.lastname: Asfour
authors.affiliation: National Register for Congenital Heart Defects, Berlin, Germany; Competence Network for Congenital Heart Defects, Berlin, Germany.
authors.forename: Ulrike M M
authors.initials: UM
authors.lastname: Bauer
authors.affiliation: National Register for Congenital Heart Defects, Berlin, Germany; Competence Network for Congenital Heart Defects, Berlin, Germany; Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany.
authors.forename: Helmut
authors.initials: H
authors.lastname: Baumgartner
authors.affiliation: National Register for Congenital Heart Defects, Berlin, Germany; Heart and Diabetes Center North Rhine-Westphalia, Center for Congenital Heart Defects, Bad Oeynhausen, Germany.
authors.forename: Deniz
authors.initials: D
authors.lastname: Kececioglu
authors.affiliation: National Register for Congenital Heart Defects, Berlin, Germany; Competence Network for Congenital Heart Defects, Berlin, Germany.
authors.forename: Marc-Andre
authors.initials: MA
authors.lastname: Körten
authors.affiliation: Competence Network for Congenital Heart Defects, Berlin, Germany; Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany.
authors.forename: Gerhard-Paul
authors.initials: GP
authors.lastname: Diller
authors.affiliation: Department of Cardiology & Angiology, Hannover Medical School, Hannover, Germany; Competence Network for Congenital Heart Defects, Berlin, Germany. Electronic address: otutarel@hotmail.com.
authors.forename: Oktay
authors.initials: O
authors.lastname: Tutarel
mesh_terms: D000328:Adult; D002423:Cause of Death / Q000639:trends; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D005858:Germany / Q000453:epidemiology; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000401:mortality*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012042:Registries
keywords: Adult congenital heart disease; Mortality; Register study