{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T18:07:41Z","timestamp":1787508461843,"version":"build-2736575974"},"reference-count":120,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2021,8,27]],"date-time":"2021-08-27T00:00:00Z","timestamp":1630022400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare and potentially lethal inherited arrhythmia disease characterized by exercise or emotion-induced bidirectional or polymorphic ventricular tachyarrhythmias. The median age of disease onset is reported to be approximately 10 years of age. The majority of CPVT patients have pathogenic variants in the gene encoding the cardiac ryanodine receptor, or calsequestrin 2. These lead to mishandling of calcium in cardiomyocytes resulting in after-depolarizations, and ventricular arrhythmias. Disease severity is particularly pronounced in younger individuals who usually present with cardiac arrest and arrhythmic syncope. Risk stratification is imprecise and long-term prognosis on therapy is unknown despite decades of research focused on pediatric CPVT populations. The purpose of this review is to summarize contemporary data on pediatric CPVT, highlight knowledge gaps and present future research directions for the clinician-scientist to address.<\/jats:p>","DOI":"10.3390\/ijms22179293","type":"journal-article","created":{"date-parts":[[2021,8,27]],"date-time":"2021-08-27T09:53:23Z","timestamp":1630058003000},"page":"9293","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia: A Translational Perspective for the Clinician-Scientist"],"prefix":"10.3390","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7438-0873","authenticated-orcid":false,"given":"Dania","family":"Kallas","sequence":"first","affiliation":[{"name":"British Columbia Children\u2019s Hospital Heart Center, 1F9-4480 Oak St., Vancouver, BC V6H 3V4, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Avani","family":"Lamba","sequence":"additional","affiliation":[{"name":"British Columbia Children\u2019s Hospital Heart Center, 1F9-4480 Oak St., Vancouver, BC V6H 3V4, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas M.","family":"Roston","sequence":"additional","affiliation":[{"name":"British Columbia Children\u2019s Hospital Heart Center, 1F9-4480 Oak St., Vancouver, BC V6H 3V4, Canada"},{"name":"Clinician-Investigator Program, University of British Columbia, 2016-1874 East Mall, Vancouver, BC V6T 1Z1, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alia","family":"Arslanova","sequence":"additional","affiliation":[{"name":"Cellular and Regenerative Medicine Centre, British Columbia Children\u2019s Hospital Research Institute, 938 W 28th Ave, Vancouver, BC V5Z 4H4, Canada"},{"name":"Molecular Cardiac Physiology Group, Department of Biomedical Physiology and Kinesiology, Simon Fraser University, 8888 University Dr., Burnaby, BC V5A 1S6, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonia","family":"Franciosi","sequence":"additional","affiliation":[{"name":"British Columbia Children\u2019s Hospital Heart Center, 1F9-4480 Oak St., Vancouver, BC V6H 3V4, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Glen F.","family":"Tibbits","sequence":"additional","affiliation":[{"name":"Cellular and Regenerative Medicine Centre, British Columbia Children\u2019s Hospital Research Institute, 938 W 28th Ave, Vancouver, BC V5Z 4H4, Canada"},{"name":"Molecular Cardiac Physiology Group, Department of Biomedical Physiology and Kinesiology, Simon Fraser University, 8888 University Dr., Burnaby, BC V5A 1S6, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shubhayan","family":"Sanatani","sequence":"additional","affiliation":[{"name":"British Columbia Children\u2019s Hospital Heart Center, 1F9-4480 Oak St., Vancouver, BC V6H 3V4, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,8,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1512","DOI":"10.1161\/01.CIR.91.5.1512","article-title":"Catecholaminergic Polymorphic Ventricular Tachycardia in Children A 7-Year Follow-up of 21 Patients","volume":"91","author":"Leenhardt","year":"1995","journal-title":"Circulation"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2035","DOI":"10.1016\/S0735-1097(99)00461-1","article-title":"Arrhythmic disorder mapped to chromosome 1q42\u2013q43 causes malignant polymorphic ventricular tachycardia in structurally normal hearts","volume":"34","author":"Swan","year":"1999","journal-title":"J. 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