{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,24]],"date-time":"2025-12-24T11:19:43Z","timestamp":1766575183399,"version":"3.48.0"},"reference-count":27,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2025,12,19]],"date-time":"2025-12-19T00:00:00Z","timestamp":1766102400000},"content-version":"vor","delay-in-days":49,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003793","name":"Swedish Heart-Lung Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003793","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,10,31]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>The prevalence of adults with congenital heart disease (ACHD) is rising due to improved paediatric care. In parallel, updated data on prognosis in adult life are needed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>The aim was to calculate the standardized mortality ratio (SMR) and death rates in ACHD compared to the general population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>Data were obtained from the national register of congenital heart disease. The general Swedish population served as a reference. SMR was calculated as the ratio between observed and expected deaths. 9089 patients (median age 28 years, interquartile range [IQR] 20\u201345, 47% females) were followed for a median of 8 years (IQR 4\u201314). 525 deaths occurred during observation. The SMR increased by lesion complexity: atrial septal defect [1.3 (95% CI: 1.1\u20131.5)]; ventricular septal defect [2.0 (1.4\u20132.7)]; congenital aortic valve disease [2.2 (1.6\u20132,9)]; Ebstein\u2019s anomaly [3.2 (1.8\u20135.2)]; tetralogy of Fallot [3.8 (2.6\u20135.2)]; congenitally corrected transposition of the great arteries [5.6 (2.9\u20139.6)]; Eisenmenger syndrome [8.7 (5.5\u201313.1)]; transposition of the great arteries with a previous atrial redirection operation [12.3 (6.8\u201320.1)]; and Fontan physiology [22.5 (12.5\u201337.0)]. Calculations were also performed by severity (mild, moderate, and severe) and age by six age groups. SMR was generally higher in younger age, and the difference in mortality from the general population was estimated to be lower for older age groups. The mortality distribution and death rate per 1000 person-years have also been calculated for each lesion.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The mortality in ACHD remains increased compared to the general population and reflects the severity of the lesion. In higher ages, the observed mortality is more in line with the general population, probably because of survival of the least affected patients, and that few persons with severe lesions have reached advanced age.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ehjopen\/oeaf165","type":"journal-article","created":{"date-parts":[[2025,12,18]],"date-time":"2025-12-18T12:57:55Z","timestamp":1766062675000},"source":"Crossref","is-referenced-by-count":0,"title":["Standardized mortality ratio in adults with congenital heart disease"],"prefix":"10.1093","volume":"5","author":[{"given":"Elisabeth","family":"Hahlin","sequence":"first","affiliation":[{"name":"Department of Diagnostics and Intervention, Ume\u00e5 University , 901 85 Ume\u00e5 ,","place":["Sweden"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9116-8084","authenticated-orcid":false,"given":"Christina","family":"Christersson","sequence":"additional","affiliation":[{"name":"Department of Medical Sciences, Cardiology, Uppsala University , 75105 Uppsala ,","place":["Sweden"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peder","family":"S\u00f6rensson","sequence":"additional","affiliation":[{"name":"Department of Medicine, Solna, Karolinska Institutet , 17177 Stockholm ,","place":["Sweden"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aleksandra","family":"Trzebiatowska-Krzynska","sequence":"additional","affiliation":[{"name":"Departments of Cardiology and Medicine and Health Sciences, Link\u00f6ping University , 58183 Link\u00f6ping ,","place":["Sweden"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zacharias","family":"Mandalenakis","sequence":"additional","affiliation":[{"name":"Department of Molecular and Clinical Medicine, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg , 40530 Gothenburg ,","place":["Sweden"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joanna","family":"Hlebowicz","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Clinical Sciences, Sk\u00e5ne University Hospital , 22100 Lund ,","place":["Sweden"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4043-7130","authenticated-orcid":false,"given":"Camilla","family":"Sandberg","sequence":"additional","affiliation":[{"name":"Department of Community Health and Rehabilitation, Ume\u00e5 University , 901 85 Ume\u00e5 ,","place":["Sweden"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0976-6910","authenticated-orcid":false,"given":"Bengt","family":"Johansson","sequence":"additional","affiliation":[{"name":"Department of Diagnostics and Intervention, Ume\u00e5 University , 901 85 Ume\u00e5 ,","place":["Sweden"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel","family":"Rinnstr\u00f6m","sequence":"additional","affiliation":[{"name":"Department of Diagnostics and Intervention, Ume\u00e5 University , 901 85 Ume\u00e5 ,","place":["Sweden"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2025,12,19]]},"reference":[{"key":"2025122406161272900_oeaf165-B1","doi-asserted-by":"crossref","first-page":"e007907","DOI":"10.1161\/JAHA.117.007907","article-title":"Adult congenital heart disease in a nationwide population 2000-2014: epidemiological trends, arrhythmia, and standardized mortality ratio","volume":"7","author":"Wu","year":"2018","journal-title":"J Am Heart Assoc"},{"key":"2025122406161272900_oeaf165-B2","doi-asserted-by":"crossref","first-page":"1149","DOI":"10.1016\/j.jacc.2010.03.085","article-title":"Changing mortality in congenital heart disease","volume":"56","author":"Khairy","year":"2010","journal-title":"J Am Coll Cardiol"},{"key":"2025122406161272900_oeaf165-B3","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1161\/CIRCULATIONAHA.106.627224","article-title":"Congenital heart disease in the general population: changing prevalence and age distribution","volume":"115","author":"Marelli","year":"2007","journal-title":"Circulation"},{"key":"2025122406161272900_oeaf165-B4","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1038\/s41569-022-00749-y","article-title":"Changing epidemiology of congenital heart disease: effect on outcomes and quality of care in adults","volume":"20","author":"Liu","year":"2023","journal-title":"Nat Rev Cardiol"},{"key":"2025122406161272900_oeaf165-B5","doi-asserted-by":"crossref","first-page":"e017704","DOI":"10.1161\/JAHA.120.017704","article-title":"Survival in children with congenital heart disease: have we reached a peak at 97%?","volume":"9","author":"Mandalenakis","year":"2020","journal-title":"J Am Heart Assoc"},{"key":"2025122406161272900_oeaf165-B6","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1016\/S0140-6736(15)60692-4","article-title":"Global, regional, and national incidence, prevalence, and years lived with disability for 301 acute and chronic diseases and injuries in 188 countries, 1990-2013: a systematic analysis for the Global Burden of Disease Study 2013","volume":"386","author":"Global Burden of Disease Study 2013 Collaborators","year":"2015","journal-title":"Lancet Lond Engl"},{"key":"2025122406161272900_oeaf165-B7","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1161\/CIRCULATIONAHA.113.008396","article-title":"Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010","volume":"130","author":"Marelli","year":"2014","journal-title":"Circulation"},{"key":"2025122406161272900_oeaf165-B8","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1186\/s12963-015-0063-z","article-title":"Projected growth of the adult congenital heart disease population in the United States to 2050: an integrative systems modeling approach","volume":"13","author":"Benziger","year":"2015","journal-title":"Popul Health Metr"},{"key":"2025122406161272900_oeaf165-B9","doi-asserted-by":"crossref","first-page":"4533","DOI":"10.1093\/eurheartj\/ehad570","article-title":"Acquired cardiovascular disease in adults with congenital heart disease: a call to action for timely preventive measures\u2014a clinical consensus statement of the European Society of Cardiology Working Group on Adult Congenital Heart Disease in collaboration with the European Association of Preventive Cardiology and the European Association of Percutaneous Cardiovascular Interventions","volume":"44","author":"Brida","year":"2023","journal-title":"Eur Heart J"},{"key":"2025122406161272900_oeaf165-B10","doi-asserted-by":"crossref","first-page":"1679","DOI":"10.1161\/CIRCULATIONAHA.109.866319","article-title":"Atrial arrhythmias in adults with congenital heart disease","volume":"120","author":"Bouchardy","year":"2009","journal-title":"Circulation"},{"key":"2025122406161272900_oeaf165-B11","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1186\/1471-2261-14-38","article-title":"Major adverse cardiovascular events in adult congenital heart disease: a population-based follow-up study from Taiwan","volume":"14","author":"Lin","year":"2014","journal-title":"BMC Cardiovasc Disord"},{"key":"2025122406161272900_oeaf165-B12","doi-asserted-by":"crossref","first-page":"586","DOI":"10.1093\/eurheartj\/ehx655","article-title":"Quantifying infective endocarditis risk in patients with predisposing cardiac conditions","volume":"39","author":"Thornhill","year":"2018","journal-title":"Eur Heart J"},{"key":"2025122406161272900_oeaf165-B13","first-page":"2048","article-title":"Incidence, risk factors, and predictors of infective endocarditis in adult congenital heart disease: focus on the use of prosthetic material","volume":"38","author":"Kuijpers","year":"2017","journal-title":"Eur Heart J"},{"key":"2025122406161272900_oeaf165-B14","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.ahj.2023.01.012","article-title":"Long-term incidence of infective endocarditis among patients with congenital heart disease","volume":"259","author":"Havers-Borgersen","year":"2023","journal-title":"Am Heart J"},{"key":"2025122406161272900_oeaf165-B15","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1016\/j.ijcard.2014.04.072","article-title":"Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification","volume":"174","author":"van Riel","year":"2014","journal-title":"Int J Cardiol"},{"key":"2025122406161272900_oeaf165-B16","doi-asserted-by":"crossref","first-page":"1610","DOI":"10.1016\/j.amjcard.2018.02.051","article-title":"Incidence and mortality of adults with pulmonary hypertension and congenital heart disease","volume":"121","author":"Schwartz","year":"2018","journal-title":"Am J Cardiol"},{"key":"2025122406161272900_oeaf165-B17","doi-asserted-by":"crossref","first-page":"e020939","DOI":"10.1161\/JAHA.120.020939","article-title":"Burden of ischemic stroke in patients with congenital heart disease: a nationwide, case-control study","volume":"10","author":"Giang","year":"2021","journal-title":"J Am Heart Assoc"},{"key":"2025122406161272900_oeaf165-B18","doi-asserted-by":"crossref","first-page":"1912","DOI":"10.1161\/CIRCULATIONAHA.117.029686","article-title":"Risk of dementia in adults with congenital heart disease: population-based cohort study","volume":"137","author":"Bagge","year":"2018","journal-title":"Circulation"},{"key":"2025122406161272900_oeaf165-B19","doi-asserted-by":"crossref","first-page":"e196762","DOI":"10.1001\/jamanetworkopen.2019.6762","article-title":"Risk of cancer among children and young adults with congenital heart disease compared with healthy controls","volume":"2","author":"Mandalenakis","year":"2019","journal-title":"JAMA Netw Open"},{"key":"2025122406161272900_oeaf165-B20","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1017\/S1047951112002144","article-title":"Cancer risk among patients with congenital heart defects: a nationwide follow-up study","volume":"24","author":"Olsen","year":"2014","journal-title":"Cardiol Young"},{"key":"2025122406161272900_oeaf165-B21","doi-asserted-by":"crossref","first-page":"1251","DOI":"10.33963\/KP.a2021.0129","article-title":"The burden of cardiovascular risk factors among seniors with congenital heart disease: a single tertiary center experience","volume":"79","author":"Kwiatek-Wrzosek","year":"2021","journal-title":"Pol Heart J Kardiologia Pol"},{"key":"2025122406161272900_oeaf165-B22","doi-asserted-by":"crossref","first-page":"131833","DOI":"10.1016\/j.ijcard.2024.131833","article-title":"Incidence of diabetes mellitus and effect on mortality in adults with congenital heart disease","volume":"401","author":"Bj\u00f6rk","year":"2024","journal-title":"Int J Cardiol"},{"key":"2025122406161272900_oeaf165-B23","doi-asserted-by":"crossref","first-page":"e014820","DOI":"10.1161\/JAHA.119.014820","article-title":"Prevalence and prognostic association of a clinical diagnosis of depression in adult congenital heart disease: results of the Boston adult congenital heart disease biobank","volume":"9","author":"Carazo","year":"2020","journal-title":"J Am Heart Assoc Cardiovasc Cerebrovasc Dis"},{"author":"Max Planck Institute for demographic Research (Germany), University of California (USA), Berkely (USA), French Institute for Demographic studies (France)","key":"2025122406161272900_oeaf165-B24"},{"key":"2025122406161272900_oeaf165-B25","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1093\/eurheartj\/ehaa554","article-title":"2020 ESC Guidelines for the management of adult congenital heart disease: The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Adult Congenital Heart Disease (ISACHD)","volume":"42","author":"Baumgartner","year":"2021","journal-title":"Eur Heart J"},{"key":"2025122406161272900_oeaf165-B26","first-page":"437","article-title":"Fiducial limits for the Poisson distribution","volume":"28","author":"Garwood","year":"1936","journal-title":"Biometrika"},{"key":"2025122406161272900_oeaf165-B27","doi-asserted-by":"crossref","first-page":"2118","DOI":"10.1161\/CIRCULATIONAHA.115.017202","article-title":"Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre","volume":"132","author":"Diller","year":"2015","journal-title":"Circulation"}],"container-title":["European Heart Journal Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ehjopen\/advance-article-pdf\/doi\/10.1093\/ehjopen\/oeaf165\/66026491\/oeaf165.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ehjopen\/article-pdf\/5\/6\/oeaf165\/66026491\/oeaf165.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ehjopen\/article-pdf\/5\/6\/oeaf165\/66026491\/oeaf165.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,24]],"date-time":"2025-12-24T11:16:20Z","timestamp":1766574980000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ehjopen\/article\/doi\/10.1093\/ehjopen\/oeaf165\/8384057"}},"subtitle":[],"editor":[{"given":"Annette Schophuus","family":"Jensen","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2025,10,31]]},"references-count":27,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2025,10,31]]}},"URL":"https:\/\/doi.org\/10.1093\/ehjopen\/oeaf165","relation":{},"ISSN":["2752-4191"],"issn-type":[{"type":"electronic","value":"2752-4191"}],"subject":[],"published-other":{"date-parts":[[2025,11]]},"published":{"date-parts":[[2025,10,31]]},"article-number":"oeaf165"}}