title: Causes of death and cardiovascular complications in adolescents and adults with congenitally malformed hearts: an autopsy study of 102 cases.
abstract: OBJECTIVE
  To identify the causes of death and main cardiovascular complications in adolescents and adults with congenitally malformed hearts.
  
  
  METHODS
  Retrospective review of 102 necropsy reports from a tertiary centre obtained over a period of 19 years.
  
  
  METHODS
  The diagnosis, the operated or non-operated state of the main defect, the cause of death, and main complications were related to the age and gender. Other clinically relevant conditions, and identifiable sequels of previous diseases, were also noted.
  
  
  RESULTS
  The ages ranged from 15 to 69 years, with a mean of 31.1 and a median of 28 years, with no difference detected according to the gender. Of the patients, two-thirds had been submitted to at least one cardiac surgery. The mean age of death was significantly higher in non-operated patients (p = 0.003). The most prevalent cause of death in the whole group was related to recent surgery, found in one-third. From them, two-fifths corresponded to reoperations. Among the others, cardiac failure was the main terminal cause in another third, and the second cause was pulmonary thromboembolism in just over one-fifth, presenting a significant association with histopathological signs of pulmonary hypertension (p = 0.011). Infection was the cause of death in 7.8% of the patients, all previously operated. Acute infective endocarditis was present or was the indication for the recent surgery in one-tenth of the patients, this cohort having a mean age of 27.8 years. There was a statistically significant association between the occurrence of endocarditis and defects causing low pulmonary blood flow (p = 0.043).
  
  
  CONCLUSIONS
  Data derived from necropsies of adults with congenital heart defects can help the multidisciplinary team refine both their diagnosis and treatment.
authors.affiliation: Laboratory of Pathology, Heart Institute (InCor), University of São Paulo Medical School, São Paulo, Brazil. vera.aiello@incor.usp.br
authors.forename: Vera Demarchi
authors.initials: VD
authors.lastname: Aiello
authors.forename: Maria Angélica
authors.initials: MA
authors.lastname: Binotto
authors.forename: Lea Maria
authors.initials: LM
authors.lastname: Demarchi
authors.forename: Antonio Augusto
authors.initials: AA
authors.lastname: Lopes
authors.forename: Miguel Barbero
authors.initials: MB
authors.lastname: Marcial
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002318:Cardiovascular Diseases / Q000209:etiology* / Q000401:mortality*; D002423:Cause of Death; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000401:mortality*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult