Hostname: page-component-cd9895bd7-lnqnp Total loading time: 0 Render date: 2024-12-18T22:21:10.475Z Has data issue: false hasContentIssue false
  • English
  • Français

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]
Get access Rights & Permissions [Opens in a new window]

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Gatzoulis, MA. Adult congenital heart disease: education, education, education. Nat Clin Pract Cardiovasc Med 2006; 3: 2–3.CrossRefGoogle ScholarPubMed
2. Oechslin, EN, Harrison, DA, Connelly, MS, Webb, GD, Siu, SC. Mode of death in adults with congenital heart disease. Am J Cardiol 2000; 86: 1111–1116.CrossRefGoogle ScholarPubMed
3. Gerlis, LM, Ho, SY, Somerville, J. A postmortem review of congenital cardiac malformations in a series of 180 adults, over the age of 16 years, born between 1865 and 1980. Cardiovasc Pathol 1999; 8: 263–272.CrossRefGoogle Scholar
4. Matsuura, K, Akizuki, S, Nakamura, N, Ishibashi-Ueda, H, Moriyama, M. A case of right isomerism showing long survival without surgery. South Med J 2007; 100: 218–221.CrossRefGoogle ScholarPubMed
5. Report of the British Cardiac Society Working Party. Grown-up congenital heart (GUCH) disease: current needs and provision of service for adolescents and adults with congenital heart disease in the UK. Heart 2002; 88 Suppl 1: i1–i14.CrossRefGoogle Scholar
6. Gutierrez, PS, Binotto, MA, Aiello, VD, Mansur, AJ. Chest pain in an adult with truncus arteriosus communis. Am J Cardiol 2004; 93: 272–273.CrossRefGoogle Scholar
7. Aiello, VD, Debich-Spicer, D, Anderson, RH. Is there still a role for cardiac autopsy in 2007? Cardiol Young 2007; 17 Suppl 2: 97–103.CrossRefGoogle Scholar
8.DATASUS- Morbidade Hospitalar. Available at: http://tabnet.datasus.gov.br/cgi/deftohtm.exe?sih/cnv/miuf.def. Accessed October 17, 2008.Google Scholar
9. Bolger, AP, Coats, AJ, Gatzoulis, MA. Congenital heart disease: the original heart failure syndrome. Eur Heart J 2003; 24: 970–976.CrossRefGoogle ScholarPubMed
10. Chowdhury, UK, Sathia, S, Ray, R, Singh, R, Pradeep, KK, Venugopal, P. Histopathology of the right ventricular outflow tract and its relationship to clinical outcomes and arrhythmias in patients with tetralogy of Fallot. J Thorac Cardiovasc Surg 2006; 132: 270–277.CrossRefGoogle ScholarPubMed
11. Babu-Narayan, SV, Kilner, PJ, Li, W, et al. Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of Fallot and its relationship to adverse markers of clinical outcome. Circulation 2006; 113: 405–413.CrossRefGoogle ScholarPubMed
12. Aiello, VD, Binotto, MA. Myocardial remodeling in congenital heart disease. Arq Bras Cardiol 2007; 88: e185–e186.CrossRefGoogle ScholarPubMed
13. Daliento, L, Somerville, J, Presbitero, P, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998; 19: 1845–1855.CrossRefGoogle ScholarPubMed
14. Caramuru, LH, Maeda, NY, Bydlowski, SP, Lopes, AA. Age-dependent likelihood of in situ thrombosis in secondary pulmonary hypertension. Clin Appl Thromb Hemost 2004; 10: 217–223.CrossRefGoogle ScholarPubMed
15. Li, W, Somerville, J. Infective endocarditis in the grown-up congenital heart (GUCH) population. Eur Heart J 1998; 19: 166–173.CrossRefGoogle ScholarPubMed
16. Knirsch, W, Haas, NA, Uhlemann, F, Dietz, K, Lange, PE. Clinical course and complications of infective endocarditis in patients growing up with congenital heart disease. Int J Cardiol 2005; 101: 285–291.CrossRefGoogle ScholarPubMed
17. Westaby, S, Evans, BJ, Ormerod, O. Pulmonary artery dissection in patients with Eisenmenger’s syndrome. N Engl J Med 2007; 356: 2110–2112.CrossRefGoogle ScholarPubMed
18. Somerville, J. The Denolin Lecture: The woman with congenital heart disease. Eur Heart J 1998; 19: 1766–1775.CrossRefGoogle ScholarPubMed
19. Presbitero, P, Somerville, J, Stone, S, Aruta, E, Spiegelhalter, D, Rabajoli, F. Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus. Circulation 1994; 89: 2673–2676.Google ScholarPubMed
20. Mansur, AJ, Grinberg, M, Lopes, EA, et al. Acute rheumatic involvement of the 4 cardiac valves in a patient with tetralogy of Fallot. Arq Bras de Cardiol 1980; 35: 499–502.Google Scholar