{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T22:17:22Z","timestamp":1759961842555},"reference-count":6,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2020,11,5]],"date-time":"2020-11-05T00:00:00Z","timestamp":1604534400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background\u2003<\/jats:title>\n                  <jats:p>Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a severe genetic arrhythmogenic disorder characterized by adrenergically induced ventricular tachycardia manifesting as stress-induced syncope and sudden cardiac death. While CPVT is not associated with dilated cardiomyopathy (DCM) in most cases, the combination of both disease entities poses a major diagnostic and therapeutic challenge.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary\u2003<\/jats:title>\n                  <jats:p>We present the case of a young woman with CPVT. The clinical course since childhood was characterized by repetitive episodes of exercise-induced ventricular arrhythmias and a brady-tachy syndrome due to rapid paroxysmal atrial fibrillation and sinus bradycardia. Medical treatment included propranolol and flecainide until echocardiography showed a dilated left ventricle with severely depressed ejection fraction when the patient was 32\u2009years old. Cardiac magnetic resonance imaging revealed non-specific late gadolinium enhancement. Myocardial inflammation, however, was excluded by subsequent endomyocardial biopsy. Genetic analysis confirmed a mutation in the cardiac ryanodine receptor but no pathogenetic variant associated with DCM. Guideline-directed medical therapy for HFrEF was limited due to symptomatic hypotension. Over the next months, the patient developed progressive heart failure symptoms that were finally managed by heart transplantation.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion\u2003<\/jats:title>\n                  <jats:p>Management in patients with CPVT and DCM is challenging, as Class I antiarrhythmic drugs are not recommended in structural heart disease and prophylactic internal cardioverter-defibrillator implantation without adjuvant antiarrhythmic therapy can be detrimental. Regular echocardiographic screening for DCM is recommendable in patients with CPVT. A multidisciplinary team of heart failure specialists, electrophysiologists, geneticists, and imaging specialists is needed to collaborate in the delivery of clinical care.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytaa299","type":"journal-article","created":{"date-parts":[[2020,11,5]],"date-time":"2020-11-05T06:31:22Z","timestamp":1604557882000},"page":"1-6","source":"Crossref","is-referenced-by-count":4,"title":["Catecholaminergic polymorphic ventricular tachycardia complicated by dilated cardiomyopathy: a case report"],"prefix":"10.1093","volume":"4","author":[{"given":"Granitz","family":"Christina","sequence":"first","affiliation":[{"name":"Clinic of Internal Medicine II, Department of Cardiology, Paracelsus Medical University of Salzburg, M\u00fcllner Hauptstrasse 48, 5020 Salzburg, Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jirak","family":"Peter","sequence":"additional","affiliation":[{"name":"Clinic of Internal Medicine II, Department of Cardiology, Paracelsus Medical University of Salzburg, M\u00fcllner Hauptstrasse 48, 5020 Salzburg, Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Strohmer","family":"Bernhard","sequence":"additional","affiliation":[{"name":"Clinic of Internal Medicine II, Department of Cardiology, Paracelsus Medical University of Salzburg, M\u00fcllner Hauptstrasse 48, 5020 Salzburg, Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P\u00f6lzl","family":"Gerhard","sequence":"additional","affiliation":[{"name":"Clinic of Internal Medicine III, Department of Cardiology and Angiology, University Hospital Innsbruck, Anichstra\u00dfe 35, 6020 Innsbruck, Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2020,11,5]]},"reference":[{"key":"2021010812212392900_ytaa299-B1","doi-asserted-by":"crossref","first-page":"92","DOI":"10.3389\/fcvm.2019.00092","article-title":"CPVT: arrhythmogenesis, therapeutic management, and future perspectives. A brief review of the literature","volume":"6","author":"Baltogiannis","year":"2019","journal-title":"Front Cardiovasc Med"},{"key":"2021010812212392900_ytaa299-B2","doi-asserted-by":"crossref","first-page":"2793","DOI":"10.1093\/eurheartj\/ehv316","article-title":"2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: the Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC)","volume":"36","author":"Priori","year":"2015","journal-title":"Eur Heart J"},{"key":"2021010812212392900_ytaa299-B3","doi-asserted-by":"crossref","first-page":"2129","DOI":"10.1093\/eurheartj\/ehw128","article-title":"2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure","volume":"37","author":"Ponikowski","year":"2016","journal-title":"Eur Heart J"},{"key":"2021010812212392900_ytaa299-B4","doi-asserted-by":"crossref","first-page":"1791","DOI":"10.1016\/j.hrthm.2018.06.046","article-title":"Implantable cardioverter-defibrillator use in catecholaminergic polymorphic ventricular tachycardia: a systematic review","volume":"15","author":"Roston","year":"2018","journal-title":"Heart Rhythm"},{"key":"2021010812212392900_ytaa299-B5","doi-asserted-by":"crossref","first-page":"1330","DOI":"10.1161\/CIRCULATIONAHA.117.031947","article-title":"RBM20 mutations induce an arrhythmogenic dilated cardiomyopathy related to disturbed calcium handling","volume":"138","author":"van den Hoogenhof","year":"2018","journal-title":"Circulation"},{"key":"2021010812212392900_ytaa299-B6","doi-asserted-by":"crossref","first-page":"151","DOI":"10.2174\/157340641202160209094548","article-title":"Calcium homeostasis and kinetics in heart failure","volume":"12","author":"Davlouros","year":"2016","journal-title":"Med Chem"}],"container-title":["European Heart Journal - Case Reports"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/ehjcr\/article-pdf\/4\/6\/1\/35544585\/ytaa299.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/ehjcr\/article-pdf\/4\/6\/1\/35544585\/ytaa299.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,1,13]],"date-time":"2021-01-13T23:52:45Z","timestamp":1610581965000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ehjcr\/article\/4\/6\/1\/5956363"}},"subtitle":[],"editor":[{"given":"Christian Fielder","family":"Camm","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2020,11,5]]},"references-count":6,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2020,11,5]]},"published-print":{"date-parts":[[2020,12,1]]}},"URL":"https:\/\/doi.org\/10.1093\/ehjcr\/ytaa299","relation":{},"ISSN":["2514-2119"],"issn-type":[{"value":"2514-2119","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2020,12]]},"published":{"date-parts":[[2020,11,5]]}}}