{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T13:23:24Z","timestamp":1793885004200,"version":"4.8.0"},"reference-count":24,"publisher":"Oxford University Press (OUP)","issue":"37","license":[{"start":{"date-parts":[[2019,6,23]],"date-time":"2019-06-23T00:00:00Z","timestamp":1561248000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Finnish government"},{"DOI":"10.13039\/501100005633","name":"Finnish Foundation for Cardiovascular Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005633","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008723","name":"Finnish Medical Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008723","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,10,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aims<\/jats:title><jats:p>The present study was done to assess the role of sudden cardiac death (SCD) among the presenting manifestations of and fatalities from cardiac sarcoidosis (CS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>We analysed altogether 351 cases of CS presenting from year 1998 through 2015 in Finland. There were 262 patients with a clinical diagnosis and treatment of CS, 27 patients with an initial lifetime diagnosis of giant cell myocarditis that was later converted to CS, and 62 cases detected at autopsy and identified by screening &amp;gt;820\u00a0000 death certificates from the national cause-of-death registry. The total case series comprised 253 females and 98 males aged on average 52\u2009years at presentation. High-grade atrioventricular block was the most common first sign of CS (n\u2009=\u2009147, 42%) followed by heart failure (n\u2009=\u200958, 17%), unexpected fatal (n\u2009=\u200938) or aborted (n\u2009=\u200912) SCD (14%), and sustained ventricular tachycardia (n\u2009=\u200948, 14%). Severe coronary artery disease was found at autopsy concomitant with CS in four of the 38 cases presenting with fatal SCD. Of all deaths recorded till the end of 2015, 64% (n\u2009=\u200954\/84) were unexpected SCDs from CS that had either been silent during life or defied all attempts at diagnosis. The Kaplan\u2013Meier estimate (95% CI) of survival from symptom onset was 85% (80\u201390%) at 5\u2009years and 76% (68\u201384%) at 10\u2009years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Together fatal and aborted SCD constitute 14% of the presenting manifestations of CS. Nearly two-thirds of all fatalities from CS are caused by undiagnosed granulomas in the heart.<\/jats:p><jats:p\/><\/jats:sec>","DOI":"10.1093\/eurheartj\/ehz428","type":"journal-article","created":{"date-parts":[[2019,5,30]],"date-time":"2019-05-30T11:08:51Z","timestamp":1559214531000},"page":"3121-3128","source":"Crossref","is-referenced-by-count":166,"title":["Sudden death in cardiac sarcoidosis: an analysis of nationwide clinical and cause-of-death registries"],"prefix":"10.1093","volume":"40","author":[{"given":"Kaj","family":"Ekstr\u00f6m","sequence":"first","affiliation":[{"name":"Helsinki University Hospital Heart and Lung Center, Helsinki University and , Stenb\u00e4ckinkatu 9, Helsinki, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jukka","family":"Lehtonen","sequence":"additional","affiliation":[{"name":"Helsinki University Hospital Heart and Lung Center, Helsinki University and , Stenb\u00e4ckinkatu 9, Helsinki, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanna-Kaisa","family":"Nordenswan","sequence":"additional","affiliation":[{"name":"Helsinki University Hospital Heart and Lung Center, Helsinki University and , Stenb\u00e4ckinkatu 9, Helsinki, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikko I","family":"M\u00e4yr\u00e4np\u00e4\u00e4","sequence":"additional","affiliation":[{"name":"Helsinki University Hospital Pathology, Helsinki University and , Haartmaninkatu 3, Helsinki, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"R\u00e4is\u00e4nen-Sokolowski","sequence":"additional","affiliation":[{"name":"Helsinki University Hospital Pathology, Helsinki University and , Haartmaninkatu 3, Helsinki, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1161-2470","authenticated-orcid":false,"given":"Riina","family":"Kandolin","sequence":"additional","affiliation":[{"name":"Helsinki University Hospital Heart and Lung Center, Helsinki University and , Stenb\u00e4ckinkatu 9, Helsinki, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piia","family":"Simonen","sequence":"additional","affiliation":[{"name":"Helsinki University Hospital Heart and Lung Center, Helsinki University and , Stenb\u00e4ckinkatu 9, Helsinki, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P\u00e4ivi","family":"Pietil\u00e4-Effati","sequence":"additional","affiliation":[{"name":"Vaasa Central Hospital Department of Cardiology, , Hietalahdenkatu 2-4, Vaasa, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aleksi","family":"Alatalo","sequence":"additional","affiliation":[{"name":"Sein\u00e4joki Central Hospital Interventional Cardiac Unit, The Hospital District of South Ostrobothnia, , Hanneksenrinne 7, Sein\u00e4joki, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seppo","family":"Utriainen","sequence":"additional","affiliation":[{"name":"South Karelia Central Hospital Department of Internal Medicine, , Valto K\u00e4kel\u00e4n katu 1, 53130 Lappeenranta, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tuomas T","family":"Rissanen","sequence":"additional","affiliation":[{"name":"North Karelia Central Hospital Heart Center, , Tikkam\u00e4entie 16, Joensuu, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petri","family":"Haataja","sequence":"additional","affiliation":[{"name":"Tays Heart Hospital , Department of Cardiology, Ensitie 4, 33560 Tampere, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorma","family":"Kokkonen","sequence":"additional","affiliation":[{"name":"Central Finland Central Hospital Department of Internal Medicine, , Keskussairaalantie 19, Jyv\u00e4skyl\u00e4, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tapani","family":"Vihinen","sequence":"additional","affiliation":[{"name":"Turku University Hospital and University of Turku Heart Center, , H\u00e4meentie 11, Turku, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heikki","family":"Miettinen","sequence":"additional","affiliation":[{"name":"Kuopio University Hospital Heart Center, , Puijonlaaksontie 2, Kuopio, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kari","family":"Kaikkonen","sequence":"additional","affiliation":[{"name":"University Hospital of Oulu and University of Oulu Research Unit of Internal Medicine, , Kajaanintie 50, Oulu, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tuomas","family":"Kerola","sequence":"additional","affiliation":[{"name":"P\u00e4ij\u00e4t-H\u00e4me Central Hospital Department of Internal Medicine, , Keskussairaalankatu 7, Lahti, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markku","family":"Kupari","sequence":"additional","affiliation":[{"name":"Helsinki University Hospital Heart and Lung Center, Helsinki University and , Stenb\u00e4ckinkatu 9, Helsinki, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2019,6,23]]},"reference":[{"key":"2022102609303311100_ehz428-B1","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1016\/0002-9343(77)90121-8","article-title":"Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients (group 1) and review of 78 previously described necropsy patients (group 2)","volume":"63","author":"Roberts","year":"1977","journal-title":"Am J Med"},{"key":"2022102609303311100_ehz428-B2","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1111\/j.1749-6632.1986.tb18531.x","article-title":"The prognosis of sarcoid heart disease in the United Kingdom","volume":"465","author":"Fleming","year":"1986","journal-title":"Ann NY Acad Sci"},{"key":"2022102609303311100_ehz428-B3","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1093\/qjmed\/hcg038","article-title":"Sudden, unexpected cardiac or unexplained death in England: a national survey","volume":"96","author":"Bowker","year":"2003","journal-title":"Q J Med"},{"key":"2022102609303311100_ehz428-B4","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1136\/hrt.2004.045518","article-title":"Sudden adult death syndrome and other nonischaemic causes of sudden cardiac death","volume":"92","author":"Fabre","year":"2005","journal-title":"Heart"},{"key":"2022102609303311100_ehz428-B5","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1007\/s00428-010-1003-8","article-title":"Cardiac sarcoidosis and sudden death. The heart may look normal or mimic other cardiomyopathies","volume":"458","author":"Bagwan","year":"2011","journal-title":"Virchows Arch"},{"key":"2022102609303311100_ehz428-B6","first-page":"2663","article-title":"Cardiac manifestations of sarcoidosis: diagnosis and management","volume":"38","author":"Birnie","year":"2017","journal-title":"Eur Heart J"},{"key":"2022102609303311100_ehz428-B7","doi-asserted-by":"crossref","first-page":"624","DOI":"10.1161\/CIRCULATIONAHA.114.011522","article-title":"Cardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study","volume":"131","author":"Kandolin","year":"2015","journal-title":"Circulation"},{"key":"2022102609303311100_ehz428-B8","doi-asserted-by":"crossref","first-page":"1305","DOI":"10.1016\/j.hrthm.2014.03.043","article-title":"HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis","volume":"11","author":"Birnie","year":"2014","journal-title":"Heart Rhythm"},{"key":"2022102609303311100_ehz428-B9","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1111\/j.1365-2796.2011.02396.x","article-title":"Diagnosing isolated cardiac sarcoidosis","volume":"270","author":"Kandolin","year":"2011","journal-title":"J Intern Med"},{"key":"2022102609303311100_ehz428-B10","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.ahj.2008.09.009","article-title":"Cardiac sarcoidosis","volume":"157","author":"Kim","year":"2009","journal-title":"Am Heart J"},{"key":"2022102609303311100_ehz428-B11","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1136\/postgradmedj-2014-133219","article-title":"Cardiac sarcoidosis: diagnosis and management","volume":"91","author":"Dubrey","year":"2015","journal-title":"Postgrad Med J"},{"key":"2022102609303311100_ehz428-B12","doi-asserted-by":"crossref","first-page":"1570","DOI":"10.1016\/j.hrthm.2011.06.031","article-title":"Causes of nonischemic sudden cardiac death in the current era","volume":"8","author":"Hookana","year":"2011","journal-title":"Heart Rhythm"},{"key":"2022102609303311100_ehz428-B13","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1016\/S0735-1097(02)02715-8","article-title":"A clinical and histopathologic comparison of cardiac sarcoidosis and idiopathic giant cell myocarditis","volume":"41","author":"Okura","year":"2003","journal-title":"J Am Coll Cardiol"},{"key":"2022102609303311100_ehz428-B14","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1016\/S0002-9149(01)01978-6","article-title":"Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone","volume":"88","author":"Yazaki","year":"2001","journal-title":"Am J Cardiol"},{"key":"2022102609303311100_ehz428-B15","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1016\/j.acvd.2016.12.014","article-title":"Cardiac sarcoidosis: diagnosis, therapeutic management and prognostic factors","volume":"110","author":"Chapelon-Abric","year":"2017","journal-title":"Arch Cardiovasc Dis"},{"key":"2022102609303311100_ehz428-B16","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1016\/j.chest.2016.08.1457","article-title":"Cardiac sarcoidosis. The impact of age and implanted devices on survival","volume":"151","author":"Zhou","year":"2017","journal-title":"Chest"},{"key":"2022102609303311100_ehz428-B17"},{"key":"2022102609303311100_ehz428-B18","doi-asserted-by":"crossref","first-page":"e211","DOI":"10.1161\/CIRCULATIONAHA.115.016258","article-title":"Letter by Al-Kindi and Oliveira regarding article: \u201cCardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study\u201d","volume":"132","author":"Al-Kindi","year":"2015","journal-title":"Circulation"},{"key":"2022102609303311100_ehz428-B19","doi-asserted-by":"crossref","first-page":"1739","DOI":"10.1253\/circj.CJ-17-0701","article-title":"Trends in hospitalizations for cardiac sarcoidosis in the United States, 2005-2011","volume":"81","author":"Guo","year":"2017","journal-title":"Circ J"},{"key":"2022102609303311100_ehz428-B20","doi-asserted-by":"crossref","first-page":"1690","DOI":"10.1183\/13993003.00477-2016","article-title":"Sarcoidosis incidence and prevalence: a nationwide register-based assessment in Sweden","volume":"48","author":"Arkema","year":"2016","journal-title":"Eur Respir J"},{"key":"2022102609303311100_ehz428-B21","doi-asserted-by":"crossref","first-page":"e003040.","DOI":"10.1161\/JAHA.115.003040","article-title":"Magnetic resonance imaging as a predictor of survival free of life-threatening arrhythmias and transplantation in cardiac sarcoidosis","volume":"5","author":"Ekstr\u00f6m","year":"2016","journal-title":"J Am Heart Assoc"},{"key":"2022102609303311100_ehz428-B22","doi-asserted-by":"crossref","first-page":"e006145.","DOI":"10.1161\/CIRCEP.117.006145","article-title":"Outcome of cardiac sarcoidosis presenting with high-grade atrioventricular block","volume":"11","author":"Nordenswan","year":"2018","journal-title":"Circ Arrhythm Electrophysiol"},{"key":"2022102609303311100_ehz428-B23","first-page":"318","article-title":"2017 AHA\/ACC\/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology Foundation\/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society","volume":"25","author":"Al-Khatib","year":"2017","journal-title":"J Am Coll Cardiol"},{"key":"2022102609303311100_ehz428-B24","doi-asserted-by":"crossref","first-page":"e006685.","DOI":"10.1161\/CIRCEP.118.006685","article-title":"Which patients with cardiac sarcoidosis should receive implantable cardioverter-defibrillators","volume":"11","author":"Birnie","year":"2018","journal-title":"Circ Arrhythm Electrophysiol"}],"container-title":["European Heart Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/eurheartj\/article-pdf\/40\/37\/3121\/46640509\/eurheartj_40_37_3121.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/eurheartj\/article-pdf\/40\/37\/3121\/46640509\/eurheartj_40_37_3121.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,17]],"date-time":"2023-09-17T01:40:17Z","timestamp":1694914817000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/eurheartj\/article\/40\/37\/3121\/5522188"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,6,23]]},"references-count":24,"journal-issue":{"issue":"37","published-online":{"date-parts":[[2019,6,23]]},"published-print":{"date-parts":[[2019,10,1]]}},"URL":"https:\/\/doi.org\/10.1093\/eurheartj\/ehz428","relation":{},"ISSN":["0195-668X","1522-9645"],"issn-type":[{"value":"0195-668X","type":"print"},{"value":"1522-9645","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2019,10,1]]},"published":{"date-parts":[[2019,6,23]]}}}