Abstract
Although the epidemiology of congenital heart disease (CHD) has been described, the authors believe changes in prenatal factors such as termination of pregnancy for fetal anomaly and prenatal vitamin supplementation have altered the birth prevalence of severe CHD. This population-based study reviewed the Nationwide Inpatient Sample (NIS) database and identified all cases with a severe CHD diagnosis among all live birth entries from 1999 to 2008. A time trend analysis then was performed for specific severe CHD diagnoses stratified by race, socioeconomic status, and geographic location. Overall, severe CHD prevalence was 147.4 per 100,000 live births, with a temporal decrease in prevalence from 168.9 per 100,000 in 1999 to 129.3 per 100,000 in 2008 (p = 0.03). Among the 12 severe CHD diagnoses included in our cohort, the prevalence of truncus arteriosus (p = 0.02), tetralogy of Fallot (p = 0.001), hypoplastic left heart syndrome (p = 0.001), and pulmonary atresia (p = 0.01) decreased significantly during the study period. The observed prevalence trends varied significantly by race (Caucasians), socioeconomic class (upper income quartiles), and geographic location (Northeast and West regions). The study findings showed a temporal decrease in severe CHD prevalence, which varied by race, socioeconomic status, and geographic location. The authors speculated that the observed trend might be due to increased termination of fetuses with prenatally diagnosed CHD. The impact of sociodemographic variables on the observed prevalence trend might be due to differences in access to specialized perinatal care and fetal heart programs or because of variability in termination of pregnancy.
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Bjornard K, Riehle-Colarusso T, Gilboa SM, Correa A (2013) Patterns in the prevalence of congenital heart defects, metropolitan Atlanta, 1978 to 2005: birth defects research: Part A. Clin Mol Teratol 97:87–94
Botto LD, Correa A, Erickson JD (2001) Racial and temporal variations in the prevalence of heart defects. Pediatrics 107:U18–U25
Bull C, Assoc BPC (1999) Current and potential impact of fetal diagnosis on prevalence and spectrum of serious congenital heart disease at term in the Uk. Lancet 354:1242–1247
Dickinson DF, Arnold R, Wilkinson JL (1981) Congenital heart disease among 160,480 liveborn children in Liverpool 1960 to 1969: implications for surgical treatment. BMJ 46:55–62
Dilber D, Malcic I (2010) Spectrum of congenital heart defects in Croatia. Eur J Pediatr 169:543–550
Ferencz C, Rubin JD, McCarter RJ, Brenner JI, Neill CA, Perry LW, Hepner SI, Downing JW (1985) Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study. Am J epidemiol 121:31–36
Fixler DE, Pastor P, Chamberlin M, Sigman E, Eifler CW (1990) Trends in congenital heart disease in Dallas County births, 1971–1984. Circulation 81:137–142
Grabitz RG, Joffres MR, Collins-Nakai RL (1988) Congenital heart disease: incidence in the first year of life. The Alberta Heritage Pediatric Cardiology Program. Am J Epidemiol 128:381–388
HCUP Nationwide Inpatient Sample (NIS) Healthcare Cost and Utilization Project (HCUP), 1998–2008. Agency for Healthcare Research and Quality. Retrieved 22 October 2013 at http://www.hcup-us.ahrq.gov/nisoverview.jsp
Hoffman JI, Christianson R (1978) Congenital heart disease in a cohort of 19,502 births with long-term follow-up. Am J Cardiol 42:641–647
Hoffman JI, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39:1890–1900
Ionescu-Ittu R, Marelli AJ, Mackie AS, Pilote L (2009) Prevalence of severe congenital heart disease after folic acid fortification of grain products: time trend analysis in Quebec, Canada. BMJ 338:b1673
Khoshnood B, De Vigan C, Vodovar V, Goujard J, Lhomme A, Bonnet D, Goffinet F (2005) Trends in prenatal diagnosis, pregnancy termination, and perinatal mortality of newborns with congenital heart disease in france, 1983–2000: a population-based evaluation. Pediatrics 115:95–101
Khoshnood B, Lelong N, Houyel L, Thieulin AC, Jouannic JM, Magnier S, Delezoide AL, Magny JF, Rambaud C, Bonnet D, Goffinet F, Group ES (2012) Prevalence, timing of diagnosis, and mortality of newborns with congenital heart defects: a population-based study. Heart 98:1667–1673
Khoshnood B, Loane M, Garne E, Addor MC, Arriola L, Bakker M, Barisic I, Bianca S, Boyd P, Calzolari E, Doray B, Draper E, Gatt M, Haeusler M, Melve KK, Latos-Bielenska A, McDonnell B, Mullaney C, Nelen V, O’Mahony M, Pierini A, Queisser-Luft A, Randrianaivo H, Rankin J, Rissmann A, Salvador J, Tucker D, Verellen-Dumoulin C, Wellesley D, Zymak-Zakutnya N, Dolk H (2013) Recent decrease in the prevalence of congenital heart defects in Europe. J Pediatr 162(108–113):e102
Lee K, Khoshnood B, Chen L, Wall SN, Cromie WJ, Mittendorf RL (2001) Infant mortality from congenital malformations in the United States, 1970–1997. Obstet Gynecol 98:620–627
Lee JE, Jung KL, Kim SE, Nam SH, Choi SJ, Oh SY, Roh CR, Kim JH (2010) Prenatal diagnosis of congenital heart disease: trends in pregnancy termination rate and perinatal and 1-year infant mortalities in Korea between 1994 and 2005. J Obstet Gynecol Res 36:474–478
Lindinger A, Schwedler G, Hense HW (2010) Prevalence of congenital heart defects in newborns in Germany: results of the first registration year of the pan study (July 2006 to June 2007). Klin Padiatr 222:321–326
McAuliffe FM, Trines J, Nield LE, Chitayat D, Jaeggi E, Hornberger LK (2005) Early fetal echocardiography: a reliable prenatal diagnosis tool. Am J Obstet Gynecol 193:1253–1259
Mitchell SC, Korones SB, Berendes HW (1971) Congenital heart disease in 56,109 births incidence and natural history. Circulation 43:323–332
Montana E, Khoury MJ, Cragan JD, Sharma S, Dhar P, Fyfe D (1996) Trends and outcomes after prenatal diagnosis of congenital cardiac malformations by fetal echocardiography in a well-defined birth population, Atlanta, Georgia, 1990–1994. J Am Coll Cardiol 28:1805–1809
Moons P, Sluysmans T, De Wolf D, Massin M, Suys B, Benatar A, Gewillig M (2009) Congenital heart disease in 111,225 births in Belgium: birth prevalence, treatment, and survival in the 21st century. Acta Paediatr 98:472–477
Nationwide Inpatient Sample (NIS) Trends Supplemental Files, 1998–2008. Retrieved 28 September 2013 at http://www.hcup-us.ahrq.gov/db/nation/nis/nistrends.jsp
Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A (2008) Prevalence of congenital heart defects in metropolitan Atlanta, 1998–2005. J Pediatr 153:807–813
Tennstedt C, Chaoui R, Korner H, Dietel M (1999) Spectrum of congenital heart defects and extracardiac malformations associated with chromosomal abnormalities: results of a seven-year necropsy study. Heart 82:34–39
van der Linde D, Konings EE, Slager MA, Witsenburg M, Helbing WA, Takkenberg JJ, Roos-Hesselink JW (2011) Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol 58:2241–2247
Wren C, Richmond S, Donaldson L (2000) Temporal variability in birth prevalence of cardiovascular malformations. Heart 83:414–419
Acknowledgments
We acknowledge the Nationwide Inpatient Sample (NIS), the Healthcare Cost and Utilization Project (HCUP), and the Agency for Healthcare Research and Quality for granting us unlimited access to their database. We also thank Jen Yau, John Doucette, and Ugochi Egbe for their contribution during data mining, formatting, statistical analysis, and proofreading.
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Egbe, A., Uppu, S., Lee, S. et al. Changing Prevalence of Severe Congenital Heart Disease: A Population-Based Study. Pediatr Cardiol 35, 1232–1238 (2014). https://doi.org/10.1007/s00246-014-0921-7
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DOI: https://doi.org/10.1007/s00246-014-0921-7