{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T15:18:24Z","timestamp":1787498304444,"version":"build-2736575974"},"reference-count":20,"publisher":"BMJ","issue":"2","license":[{"start":{"date-parts":[[2021,9,14]],"date-time":"2021-09-14T00:00:00Z","timestamp":1631577600000},"content-version":"unspecified","delay-in-days":13,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003793","name":"Swedish Heart-Lung Foundation","doi-asserted-by":"crossref","award":["20090724"],"award-info":[{"award-number":["20090724"]}],"id":[{"id":"10.13039\/501100003793","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100003793","name":"Swedish Heart-Lung Foundation","doi-asserted-by":"crossref","award":["2018\u20100589"],"award-info":[{"award-number":["2018\u20100589"]}],"id":[{"id":"10.13039\/501100003793","id-type":"DOI","asserted-by":"crossref"}]},{"name":"ALF","award":["236611"],"award-info":[{"award-number":["236611"]}]},{"name":"Hjartebarnsfonden","award":["2\/20 FO"],"award-info":[{"award-number":["2\/20 FO"]}]},{"DOI":"10.13039\/501100004359","name":"Swedish Research Council","doi-asserted-by":"crossref","award":["2\/20"],"award-info":[{"award-number":["2\/20"]}],"id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100004359","name":"Swedish Research Council","doi-asserted-by":"crossref","award":["2018-02527"],"award-info":[{"award-number":["2018-02527"]}],"id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100004359","name":"Swedish Research Council","doi-asserted-by":"crossref","award":["VRREG 2019-00193"],"award-info":[{"award-number":["VRREG 2019-00193"]}],"id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Open Heart"],"accepted":{"date-parts":[[2021,8,31]]},"published-print":{"date-parts":[[2021,9]]},"abstract":"<jats:sec>\n                    <jats:title>Background and objectives<\/jats:title>\n                    <jats:p>During the last decades, the survival rates in patients with congenital heart disease have increased dramatically, particularly in patients with complex heart malformations. However, the survival in patients with simple defects is still unknown. We aimed to determine the characteristics and the risk of mortality in patients with isolated pulmonary valve stenosis (PS).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Swedish inpatient, outpatient and cause of death registries were used to identify patients born between 1970 and 2017 with a diagnosis of PS, without any other concomitant congenital heart lesion. For each patient with PS, 10 control individuals without congenital heart disease were matched by birth year and sex from the total population registry. We used median-unbiased method and Kaplan-Meier survival analysis to examine the risk of mortality.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We included 3910 patients with PS and 38\u2009770 matched controls. The median age of diagnosis of PS was 0.7 years (IQR 0.3\u20137.0). During a median follow-up of 13.5 years (IQR 6.5\u201323.5), 88 patients with PS and 192 controls died; 500 patients with PS (12%) underwent at least one transcatheter or surgical valve intervention. The overall mortality rate was significantly higher in patients with PS compared with matched controls (HR 4.67, 95% CI 3.61 to 5.99, p=0.001). Patients with an early diagnosis of PS (0\u20131\u2009year) had the highest risk of mortality (HR 10.99, 95%\u2009CI 7.84 to 15.45).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>In this nationwide, register-based cohort study, we found that the risk of mortality in patients with PS is almost five times higher compared with matched controls. Patients with an early diagnosis of PS appears to be the most vulnerable group and the regular follow-up in tertiary congenital heart units may be the key to prevention.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/openhrt-2021-001836","type":"journal-article","created":{"date-parts":[[2021,9,14]],"date-time":"2021-09-14T12:56:13Z","timestamp":1631624173000},"page":"e001836","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":8,"title":["Long-term survival in patients with isolated pulmonary valve stenosis: a not so benign disease?"],"prefix":"10.1136","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9095-1218","authenticated-orcid":false,"given":"Kristofer","family":"Skoglund","sequence":"first","affiliation":[{"name":"Department of Molecular and Clinical Medicine, University of Gothenburg, Goteborg, Sweden"},{"name":"Department of Cardiology, Sahlgrenska University Hospital, Goteborg, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annika","family":"Rosengren","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Sahlgrenska University Hospital, Goteborg, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgios","family":"Lappas","sequence":"additional","affiliation":[{"name":"Department of Molecular and Clinical Medicine, University of Gothenburg, Goteborg, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Fedchenko","sequence":"additional","affiliation":[{"name":"Department of Molecular and Clinical Medicine, University of Gothenburg, Goteborg, Sweden"},{"name":"Department of Cardiology, Sahlgrenska University Hospital, Goteborg, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zacharias","family":"Mandalenakis","sequence":"additional","affiliation":[{"name":"Department of Molecular and Clinical Medicine, University of Gothenburg, Goteborg, Sweden"},{"name":"Department of Cardiology, Sahlgrenska University Hospital, Goteborg, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2021,9,14]]},"reference":[{"key":"2025081709360502000_8.2.e001836.1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.110.946343"},{"key":"2025081709360502000_8.2.e001836.2","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1001\/jamainternmed.2016.7765","article-title":"Survivorship in children and young adults with congenital heart disease in Sweden","volume":"177","author":"Mandalenakis","year":"2017","journal-title":"JAMA Intern Med"},{"key":"2025081709360502000_8.2.e001836.3","doi-asserted-by":"crossref","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 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