Abstract
THE POPULATION demographics of patients with severe adult congenital heart disease (ACHD) have changed significantly in this century and continue to evolve. Severe congenital heart disease (CHD) is estimated to occur in approximately 8 in every 1,000 live births. 1 Marelli A.J. Ionescu-Ittu R. Mackie A.S. et al. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014; 130: 749-756 Crossref PubMed Scopus (663) Google Scholar Because of modern advances in subspecialty medical care, survival to 18 years old is now estimated at 90% in children diagnosed with severe CHD. 2 Stout K.K. Daniels C.J. Aboulhosn J.A. et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019; 139: e637-e697 PubMed Google Scholar This significant and stepwise outcome improvement has been observed over the last 3 decades and validated in multiple populations. 1 Marelli A.J. Ionescu-Ittu R. Mackie A.S. et al. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014; 130: 749-756 Crossref PubMed Scopus (663) Google Scholar , 3 Mandalenakis Z. Rosengren A. Skoglund K. et al. Survivorship in children and young adults with congenital heart disease in Sweden. JAMA Intern Med. 2017; 177: 224-230 Crossref PubMed Scopus (81) Google Scholar As of 2010, adults were estimated to account for 66% of all severe CHD in the population, a 55% increase from 2000. 1 Marelli A.J. Ionescu-Ittu R. Mackie A.S. et al. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014; 130: 749-756 Crossref PubMed Scopus (663) Google Scholar Population models project that this growth in severe ACHD prevalence will continue through 2050 to an estimated steady state of 2.3 adults per 1,000 adults in the population. 4 Benziger C.P. Stout K. Zaragoza-Macias E. et al. Projected growth of the adult congenital heart disease population in the United States to 2050: An integrative systems modeling approach. Popul Health Metr. 2015; 13: 29 Crossref PubMed Scopus (45) Google Scholar
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