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Epidemiology of Congenital Heart Disease with Emphasis on Sex-Related Aspects

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Sex-Specific Analysis of Cardiovascular Function

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 1065))

Abstract

Gender differences in prevalence, manifestation, treatment outcomes, and prognosis have been well known for acquired heart disease such as coronary artery disease. Regarding congenital heart disease (CHD), it is recognized that the incidence of each congenital heart defect varies according to sex observed during a time span of more than 40 years. As diagnostic and surgical methods for CHD have achieved dramatic advances for the past decades, more newborns with CHD were able to survive and reach adulthood. Thereafter gender differences have begun to be reported on mortality, progress to pulmonary arterial hypertension, treatment outcomes, and prognosis in patients with CHD. However, it has been less known in the field of CHD yet, and this contribution describes information that is relatively well studied to date.

Epidemiology of congenital heart disease. Art work by Piet Michiels, Leuven, Belgium

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References

  1. Rothman KJ, Fyler DC. Sex, birth order, and maternal age characteristics of infants with congenital heart defects. Am J Epidemiol. 1976;104:527–34.

    Article  CAS  PubMed  Google Scholar 

  2. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39:1890–900.

    Article  Google Scholar 

  3. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation. 2007;115:163–72.

    Article  Google Scholar 

  4. Egbe A, Uppu S, Stroustrup A, Lee S, Ho D, Srivastava S. Incidences and sociodemographics of specific congenital heart diseases in the United States of America: an evaluation of hospital discharge diagnoses. Pediatr Cardiol. 2014;35:975–82.

    Article  PubMed  Google Scholar 

  5. Yeh SJ, Chen HC, Lu CW, Wang JK, Huang LM, Huang SC, et al. Prevalence, mortality, and the disease burden of pediatric congenital heart disease in Taiwan. Pediatr Neonatol. 2013;54:113–8.

    Article  PubMed  Google Scholar 

  6. Qu Y, Liu X, Zhuang J, Chen G, Mai J, Guo X, et al. Incidence of congenital heart disease: The 9-year experience of the Guangdong Registry of Congenital Heart Disease, China. PLoS One. 2016;11:e0159257. https://doi.org/10.1371/journal.pone.0159257.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  7. Wren C, Richmond S, Donaldson L. Temporal variability in birth prevalence of cardiovascular malformations. Heart. 2000;83:414–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Oyen N, Poulsen G, Boyd HA, Wohlfahrt J, Jensen PK, Melbye M. National time trends in congenital heart defects, Denmark, 1977–2005. Am Heart J. 2009;157:467–73.

    Article  PubMed  Google Scholar 

  9. Fixler DE, Pastor P, Chamberlin M, Sigman E, Eifler CW. Trends in congenital heart disease in Dallas County births. 1971–1984. Circulation. 1990;81:137–42.

    Article  CAS  PubMed  Google Scholar 

  10. van der Linde D, Konings EE, Slager MA, Witsenburg M, Helbing WA, Takkenberg JJ, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol. 2011;58:2241–7.

    Article  Google Scholar 

  11. Pradat P, Francannet C, Harris JA, Robert E. The epidemiology of cardiovascular defects, part I: a study based on data from three large registries of congenital malformations. Pediatr Cardiol. 2003;24:195–221.

    Article  CAS  PubMed  Google Scholar 

  12. Calzolari E, Garani G, Cocchi G, Magnani C, Rivieri F, Neville A, et al. Congenital heart defects: 15 years of experience of the Emilia-Romagna Registry (Italy). Eur J Epidemiol. 2003;18:773–80.

    Article  CAS  PubMed  Google Scholar 

  13. Egbe A, Uppu S, Lee S, Stroustrup A, Ho D, Srivastava S. Temporal variation of birth prevalence of congenital heart disease in the United States. Congenit Heart Dis. 2015;10:43–50.

    Article  PubMed  Google Scholar 

  14. Moons P, Sluysmans T, De Wolf D, Massin M, Suys B, Benatar A, et al. Congenital heart disease in 111 225 births in Belgium: birth prevalence, treatment and survival in the 21st century. Acta Paediatr. 2009;98:472–7.

    Article  PubMed  Google Scholar 

  15. Liebson PR. Cardiovascular risk in special populations IV: congenital heart defects. Prev Cardiol. 2010;13:49–55.

    Article  PubMed  Google Scholar 

  16. Aubry P, Demian H. Sex differences in congenital heart disease. Ann Cardiol Angeiol (Paris). 2016;65:440–5.

    Article  CAS  Google Scholar 

  17. McBride KL, Marengo L, Canfield M, Langlois P, Fixler D, Belmont JW. Epidemiology of noncomplex left ventricular outflow tract obstruction malformations (aortic valve stenosis, coarctation of the aorta, hypoplastic left heart syndrome) in Texas, 1999–2001. Birth Defects Res A Clin Mol Teratol. 2005;73:555–61.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Michalski AM, Richardson SD, Browne ML, Carmichael SL, Canfield MA, Van Zutphen AR, et al. Sex ratios among infants with birth defects, National Birth Defects Prevention Study, 1997–2009. Am J Med Genet. 2015;167A:1071–81.

    Article  PubMed  Google Scholar 

  19. Shaw GM, Carmichael SL, Kaidarova Z, Harris JA. Differential risks to males and females for congenital malformations among 2.5 million California births, 1989–1997. Birth Defects Res A Clin Mol Teratol. 2003;67:953–8.

    Article  CAS  PubMed  Google Scholar 

  20. Sokal R, Tata LJ, Fleming KM. Sex prevalence of major congenital anomalies in the United Kingdom: a national population-based study and international comparison meta-analysis. Birth Defects Res A Clin Mol Teratol. 2014;100:79–91.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Tennant PWG, Samarasekera SD, Pless-Mulloli T, Rankin J. Sex differences in the prevalence of congenital anomalies: a population-based study. Birth Defects Res A Clin Mol Teratol. 2011;91:894–901.

    Article  CAS  PubMed  Google Scholar 

  22. Warnes CA. Sex differences in congenital heart disease: should a woman be more like a man? Circulation. 2008;118:3–5.

    Article  PubMed  Google Scholar 

  23. Warnes CA. Bicuspid aortic valve and coarctation: two villains part of a diffuse problem. Heart. 2003;89:965–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Duffels MGJ, Engelfriet PM, Berger RMF, van Loon RLE, Hoendermis E, Vriend JWJ, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007;120:198–204.

    Article  CAS  PubMed  Google Scholar 

  25. Klitzner TS, Lee M, Rodriguez S, R-KR C. Sex-related disparity in surgical mortality among pediatric patients. Congenit Heart Dis. 2006;1:77–88.

    Article  PubMed  Google Scholar 

  26. Somerville J. The Denolin Lecture: the woman with congenital heart disease. Eur Heart J. 1998;19:1766–75.

    Article  CAS  PubMed  Google Scholar 

  27. Shapiro S, Traiger GL, Turner M, McGoon MD, Wason P, Barst RJ. Sex differences in the diagnosis, treatment and outcome of patients with pulmonary arterial hypertension enrolled in the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Chest. 2012;141:363–73.

    Article  Google Scholar 

  28. Marelli A, Gauvreau K, Landzberg M, Jenkins K. Sex differences in mortality in children undergoing congenital heart disease surgery. A United States population-based study. Circulation. 2010;122(Suppl. 1):S234–40.

    Article  PubMed  Google Scholar 

  29. Harvey RE, Coffman KE, Miller VM. Women-specific factors to consider in risk, diagnosis and treatment of cardiovascular disease. Womens Health (Lond). 2015;11:239–57.

    Article  CAS  Google Scholar 

  30. Lee HW, Eghbali-Webb M. Estrogen enhances proliferative capacity of cardiac fibroblasts by estrogen receptor- and mitogen-activated protein kinase-dependent pathways. J Mol Cell Cardiol. 1998;30:1359–68.

    Article  CAS  PubMed  Google Scholar 

  31. Fyler DC. Report of the New England regional infant cardiac program. Pediatrics. 1980;65:375–461.

    Google Scholar 

  32. DiBardino DJ, Pasquali SK, Hirsch JC, Benjamin DK, Kleeman KC, Sala-zar JD, et al. Effect of sex and race on outcome in patients undergoing congenital heart surgery: an analysis of the Society of Thoracic Surgeons Congenital Heart database. Ann Thorac Surg. 2012;94:2054–9.

    Article  PubMed  Google Scholar 

  33. Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, Vliegen HW, et al. Gender and outcome in adult congenital heart disease. Circulation. 2008;118:26–32.

    Article  PubMed  Google Scholar 

  34. Engelfriet P, Mulder BJM. Gender differences in adult congenital heart disease. Neth Heart J. 2009;17:414–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Zomer CA, Ionescu-Ittu R, Vaartjes I, Pilote L, Mackie AS, Therrien J, et al. Sex differences in hospital mortality in adults with congenital heart disease. The impact of reproductive health. J Am Coll Cardiol. 2013;62:58–67.

    Article  PubMed  Google Scholar 

  36. Sarikouch S, Boethig D, Peters B, Kropf S, Dubowy KO, Lange P, et al. Poorer right ventricular systolic function and exercise capacity in women after repair of tetralogy of Fallot: a sex comparison of standard deviation scores based on sex-specific reference values in healthy control subjects. Circ Cardiovasc Imaging. 2013;6:924–33.

    Article  PubMed  Google Scholar 

  37. Egidy Assenza G, Cassater D, Landzberg M, Geva T, Schreier J, Graham D, et al. The effects of pregnancy on right ventricular remodeling in women with repaired tetralogy of Fallot. Int J Cardiol. 2013;168:1847–52.

    Article  PubMed  Google Scholar 

  38. Sarikouch S, Koerperich H, Dubowy KO, Boethig D, Boettler P, Mir TS, et al. Impact of gender and age on cardiovascular function late after repair of tetralogy of Fallot: percentiles based on cardiac magnetic resonance. Circ Cardiovasc Imaging. 2011;4:703–11.

    Article  PubMed  Google Scholar 

  39. Kerkhof PLM, van de Ven PM, Yoo BW, Peace RA, Heyndrickx GR, Handly N. Ejection fraction as related to basic components in the left and right ventricular volume domains. Int J Cardiol. 2018;255:105–10.

    Article  PubMed  Google Scholar 

  40. Kuipers JM, van der Bom T, van Riel ACMJ, Meijboom FJ, van Dijk APJ, Pieper PG, et al. Secundum atrial septal defect is associated with reducedsurvival in adult men. Eur Heart J. 2015;36:2079–86.

    Article  Google Scholar 

  41. Yoo BW, Kim JO, Kim YJ, Choi JY, Park HK, Park YH, et al. Impact of pressure load caused by right ventricular outflow tract obstruction on right ventricular volume overload in patients with repaired tetralogy of Fallot. J Thorac Cardiovasc Surg. 2012;143:1299–304.

    Article  PubMed  Google Scholar 

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Yoo, B.W. (2018). Epidemiology of Congenital Heart Disease with Emphasis on Sex-Related Aspects. In: Kerkhof, P., Miller, V. (eds) Sex-Specific Analysis of Cardiovascular Function. Advances in Experimental Medicine and Biology, vol 1065. Springer, Cham. https://doi.org/10.1007/978-3-319-77932-4_3

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