Eisenmenger’s syndrome epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2]

Overview

The incidence and prevalence of Eisenmenger's syndrome has been decreasing gradually over the years. In the general population, the prevalence decreased from 24.6 to 11.9/million inhabitants in 2012. About 8% of patents with congenital heart diseases develop Eisenmenger's syndrome. The mortality rate of Eisenmenger's syndrome is about 27%. The risk of Eisenmenger's syndrome increases in patients with large cardiac defects or who live in developing countries due to poor healthcare access.

Epidemiology and Demographics

Incidence

  • The incidence of Eisenmenger's syndrome decreased from 2.5/million individuals/year in 1977 to 0.2/million individuals/year in 2012.[1]

Prevalence

  • Overall, about 8% of patients with congenital heart disease develop Eisenmenger's syndrome.[2]
  • About 3% of patients with a small VSD (≤1.5 cm) and 50% of patients with a large VSD (>1.5 cm) develop Eisenmenger's syndrome.[3]
  • However, the prevalence is currently decreasing due to improved detection methods of congenital heart defects.
  • In the general population, the prevalence decreased from 24.6 to 11.9/million individuals in 2012.[4]

Case-fatality rate/Mortality rate

  • At follow-up of 6 years, the mortality rate of Eisenmenger's syndrome was 27%.[5]
  • At 3, 6 and 9 years from diagnosis, the mortality rates were 27%, 50%, and 53%, respectively.[6]
  • Death usually occurs between 30 and 35 years old.

Age

  • Eisenmenger's syndrome usually manifests before puberty.
  • But it may also manifest during adolescence and early adulthood.[7]

Race

  • There is no racial predilection to Eisenmenger's syndrome.

Gender

  • Eisenmenger's syndrome affects men and women equally.

Developed Countries

The prevalence of Eisenmenger's syndrome is less common in developed countries due to better healthcare access.

Developing Countries

The prevalence of Eisenmenger's syndrome is more common in developing countries due to poor healthcare access.

References

  1. Hjortshøj CS, Jensen AS, Sørensen K, Nagy E, Johansson B, Kronvall T; et al. (2017). "Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012". Heart. 103 (17): 1353–1358. doi:10.1136/heartjnl-2016-310979. PMID 28450553.
  2. Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G; et al. (1998). "Eisenmenger syndrome. Factors relating to deterioration and death". Eur Heart J. 19 (12): 1845–55. doi:10.1053/euhj.1998.1046. PMID 9886728.
  3. Kidd L, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF, O'Fallon WM; et al. (1993). "Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects". Circulation. 87 (2 Suppl): I38–51. PMID 8425321.
  4. Hjortshøj CS, Jensen AS, Sørensen K, Nagy E, Johansson B, Kronvall T; et al. (2017). "Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012". Heart. 103 (17): 1353–1358. doi:10.1136/heartjnl-2016-310979. PMID 28450553.
  5. Hjortshøj CMS, Kempny A, Jensen AS, Sørensen K, Nagy E, Dellborg M; et al. (2017). "Past and current cause-specific mortality in Eisenmenger syndrome". Eur Heart J. 38 (26): 2060–2067. doi:10.1093/eurheartj/ehx201. PMID 28430906.
  6. Bonello B, Renard S, Mancini J, Hubert S, Habib G, Fraisse A (2014). "Life span of patients with Eisenmenger syndrome is not superior to that of patients with other causes of pulmonary hypertension". Cardiovasc Diagn Ther. 4 (5): 341–9. doi:10.3978/j.issn.2223-3652.2014.10.03. PMC 4221317. PMID 25414820.
  7. Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G; et al. (1998). "Eisenmenger syndrome. Factors relating to deterioration and death". Eur Heart J. 19 (12): 1845–55. doi:10.1053/euhj.1998.1046. PMID 9886728.



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