title: Mortality in adult congenital heart disease.
abstract: OBJECTIVE
  Mortality in adults with congenital heart disease is known to be increased, yet its extent and the major mortality risks are unclear.
  
  
  RESULTS
  The Dutch CONCOR national registry for adult congenital heart disease was linked to the national mortality registry. Cox's regression was used to assess mortality predictors. Of 6933 patients, 197 (2.8%) died during a follow-up of 24 865 patient-years. Compared with the general national population, there was excess mortality, particularly in the young. Median age at death was 48.8 years. Of all deaths, 77% had a cardiovascular origin; 45% were due to chronic heart failure (26%, age 51.0 years) or sudden death (19%, age 39.1 years). Age predicted mortality, as did gender, severity of defect, number of interventions, and number of complications [hazard ratio (HR) range 1.1-5.9, P < 0.05]. Several complications predicted all-cause mortality beyond the effects of age, gender, and congenital heart disease severity, i.e. endocarditis, supraventricular arrhythmias, ventricular arrhythmias, conduction disturbances, myocardial infarction, and pulmonary hypertension (HR range 1.4-3.1, P < 0.05). These risks were similar in patients above and below 40 years of age. Almost all complications predicted death due to heart failure (HR range 2.0-5.1, P < 0.05); conduction disturbances and pulmonary hypertension predicted sudden death (HR range 2.0-4.7, P < 0.05).
  
  
  CONCLUSIONS
  Mortality is increased in adults with congenital heart disease, particularly in the young. The vast majority die from cardiovascular causes. Mortality risk, particularly by heart failure, is increased by virtually all complications. Complications are equally hazardous in younger as in older patients.
authors.affiliation: Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Carianne L
authors.initials: CL
authors.lastname: Verheugt
authors.forename: Cuno S P M
authors.initials: CS
authors.lastname: Uiterwaal
authors.forename: Enno T
authors.initials: ET
authors.lastname: van der Velde
authors.forename: Folkert J
authors.initials: FJ
authors.lastname: Meijboom
authors.forename: Petronella G
authors.initials: PG
authors.lastname: Pieper
authors.forename: Arie P J
authors.initials: AP
authors.lastname: van Dijk
authors.forename: Hubert W
authors.initials: HW
authors.lastname: Vliegen
authors.forename: Diederick E
authors.initials: DE
authors.lastname: Grobbee
authors.forename: Barbara J M
authors.initials: BJ
authors.lastname: Mulder
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D004812:Epidemiologic Methods; D005260:Female; D006330:Heart Defects, Congenital / Q000401:mortality*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology; D017678:Sex Distribution; D055815:Young Adult