{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T19:07:53Z","timestamp":1761419273861,"version":"3.40.5"},"reference-count":16,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2019,4,17]],"date-time":"2019-04-17T00:00:00Z","timestamp":1555459200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pacing Clinical Electrophis"],"published-print":{"date-parts":[[2019,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We report a 17\u2010year\u2010old boy with a large <jats:italic>RYR2<\/jats:italic> exon 3 deletion who has a severe catecholaminergic polymorphic ventricular tachycardia (CPVT) phenotype characterized by refractoriness to both nadolol and flecainide which has previously not been reported in this subgroup of CPVT patients. Treatment options in a patient like ours are therefore limited and sympathectomy and implantable cardioverter\u2010defibrillator implantation should be considered early in the treatment course as was done in this patient. In contrast to other CPVT patients who do not usually have structural cardiac abnormalities, these patients are at a high risk of developing left ventricular noncompaction or dilated cardiomyopathy and therefore might benefit from cardiac imaging at regular intervals.<\/jats:p>","DOI":"10.1111\/pace.13668","type":"journal-article","created":{"date-parts":[[2019,3,26]],"date-time":"2019-03-26T05:00:00Z","timestamp":1553576400000},"page":"1146-1154","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["A large deletion in <i>RYR2<\/i> exon 3 is associated with nadolol and flecainide refractory catecholaminergic polymorphic ventricular tachycardia"],"prefix":"10.1111","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3410-840X","authenticated-orcid":false,"given":"Utkarsh","family":"Kohli","sequence":"first","affiliation":[{"name":"Division of Pediatric Cardiology, Department of Pediatrics Pritzker School of Medicine of the University of Chicago Chicago Illinois"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zaid","family":"Aziz","sequence":"additional","affiliation":[{"name":"Center for Arrhythmia Care, Heart &amp; Vascular Center Pritzker School of Medicine of the University of Chicago  Chicago Illinois"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew D.","family":"Beaser","sequence":"additional","affiliation":[{"name":"Center for Arrhythmia Care, Heart &amp; Vascular Center Pritzker School of Medicine of the University of Chicago  Chicago Illinois"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hemal M.","family":"Nayak","sequence":"additional","affiliation":[{"name":"Center for Arrhythmia Care, Heart &amp; Vascular Center Pritzker School of Medicine of the University of Chicago  Chicago Illinois"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,4,17]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/anec.12512"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1253\/circj.CJ-16-0326"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hrthm.2013.05.014"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.str.2011.03.016"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0095615"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hrthm.2015.09.029"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamacardio.2017.1320"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.107.711606"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.37140"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.3109\/03009734.2015.1029101"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2009.08.022"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/europace\/eut382"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2350-10-12"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCEP.112.970517"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(02)01946-0"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/pace.13446"}],"container-title":["Pacing and Clinical Electrophysiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpace.13668","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pace.13668","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/pace.13668","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pace.13668","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,9]],"date-time":"2023-09-09T14:35:57Z","timestamp":1694270157000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pace.13668"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,4,17]]},"references-count":16,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2019,8]]}},"alternative-id":["10.1111\/pace.13668"],"URL":"https:\/\/doi.org\/10.1111\/pace.13668","archive":["Portico"],"relation":{},"ISSN":["0147-8389","1540-8159"],"issn-type":[{"type":"print","value":"0147-8389"},{"type":"electronic","value":"1540-8159"}],"subject":[],"published":{"date-parts":[[2019,4,17]]},"assertion":[{"value":"2019-03-14","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-03-20","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-04-17","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}