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Causes of death and cardiovascular complications in adolescents and adults with congenitally malformed hearts: an autopsy study of 102 cases

Published online by Cambridge University Press:  27 August 2009

Vera Demarchi Aiello ,
Maria Angélica Binotto ,
Lea Maria Demarchi ,
Antonio Augusto Lopes  and
Miguel Barbero Marcial
Vera Demarchi Aiello*
Affiliation:
Laboratory of Pathology, Heart Institute (InCor), University of São Paulo Medical School, São Paulo, Brazil
Maria Angélica Binotto
Affiliation:
Clinical Unit of Paediatric Cardiology and Congenital Heart Disease in Adults, Heart Institute (InCor), University of São Paulo Medical School, São Paulo, Brazil
Lea Maria Demarchi
Affiliation:
Laboratory of Pathology, Heart Institute (InCor), University of São Paulo Medical School, São Paulo, Brazil
Antonio Augusto Lopes
Affiliation:
Clinical Unit of Paediatric Cardiology and Congenital Heart Disease in Adults, Heart Institute (InCor), University of São Paulo Medical School, São Paulo, Brazil
Miguel Barbero Marcial
Affiliation:
Division of Pediatric Surgery, Heart Institute (InCor), University of São Paulo Medical School, São Paulo, Brazil
*
Correspondence to: Vera Demarchi Aiello, Laboratory of Pathology, Heart Institute (InCor), University of São Paulo Medical School, Av. Dr Enéas C. Aguiar, 44, 05403-000 São Paulo- SP – Brazil. Tel: +55 11 3069-5252; Fax: +55 11 3069-5279; E-mail: [email protected]
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Abstract

Objectives

To identify the causes of death and main cardiovascular complications in adolescents and adults with congenitally malformed hearts.

Design

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.

Keywords

Congenital heart defectsadultscardiac surgerynecropsy
Type
Original Article
Information
Cardiology in the Young , Volume 19 , Issue 5 , October 2009 , pp. 511 - 516
Copyright
Copyright © Cambridge University Press 2009

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