{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T20:30:06Z","timestamp":1787776206425,"version":"build-2784847793"},"reference-count":17,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,5]]},"abstract":"ABSTRACT<\/jats:title>\n \n Background:<\/jats:title>\n Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited disorder characterized by ventricular arrhythmias induced by physical or emotional stress. Currently, there are limited data available on the long-term prognosis of CPVT.<\/jats:p>\n <\/jats:sec>\n \n Methods and Results:<\/jats:title>\n \n In this study, which included both retrospective and prospective components, 12 patients with CPVT (7 males and 5 females) under 18 years old were enrolled to gather and evaluate demographic, clinical, and genetic data. The mean age at diagnosis onset was 7.0 \u00b1 3.1 years. All patients experienced syncope. The mean follow-up duration was 20.1 years. During the follow-up period, all patients experienced at least one episode of supraventricular tachycardia (SVT). Despite beta-blocker therapy, nine patients experienced syncope (75%), and four patients were noncompliant with their treatment. An implantable cardiac defibrillator (ICD) implantation was performed in 10 patients (83%), and among those 5 (50%) experienced appropriate shocks. Inappropriate shocks were observed in all patients with an ICD. The left cardiac sympathetic denervation was performed in 6 patients (50%). One patient died during the follow-up period. Genetic testing was performed in eight patients, five of whom had\n RYR2<\/jats:italic>\n mutations, one patient had mutations in\n CASQ2<\/jats:italic>\n , one in\n TECRL<\/jats:italic>\n , and one was gene-elusive.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions:<\/jats:title>\n The prevalence of cardiac events, even after the initiation of beta-blocker therapy, was found to be distressingly high during long-term follow-up. SVT, such as atrial fibrillation, were found to be more common than previously thought. Combination therapy with a beta-blocker and an IC antiarrhythmic drug shows promise. An individualized approach to the selection of treatment strategies is essential.<\/jats:p>\n <\/jats:sec>","DOI":"10.4103\/apc.apc_101_24","type":"journal-article","created":{"date-parts":[[2024,10,2]],"date-time":"2024-10-02T05:00:20Z","timestamp":1727845220000},"page":"196-203","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":2,"title":["Long-term clinical course of patients with catecholaminergic polymorphic ventricular tachycardia: A more than 10-year follow-up cohort study"],"prefix":"10.4103","volume":"17","author":[{"given":"Ekaterina","family":"Kulbachinskaya","sequence":"first","affiliation":[{"name":"Department of Innovative Pediatrics and Pediatric Surgery, Veltischev Research and Clinical Institute for Pediatrics and Pediatric Surgery of the Pirogov Russian National Research Medical University, Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vera","family":"Bereznitskaya","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Veltischev Research and Clinical Institute for Pediatrics and Pediatric Surgery of the Pirogov Russian National Research Medical University, Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2024,10,1]]},"reference":[{"key":"R1-20241204","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1016\/j.hlc.2018.10.016","article-title":"An update on the diagnosis and management of catecholaminergic polymorphic ventricular tachycardia","volume":"28","author":"Pflaumer","year":"2019","journal-title":"Heart Lung Circ"},{"key":"R2-20241204","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1017\/S1047951113001091","article-title":"Complex atrial arrhythmias as first manifestation of catecholaminergic polymorphic ventricular tachycardia: An unusual course in a patient with a new mutation in ryanodine receptor type 2 gene","volume":"24","author":"Lawrenz","year":"2014","journal-title":"Cardiol Young"},{"key":"R3-20241204","doi-asserted-by":"crossref","first-page":"1606","DOI":"10.1253\/circj.71.1606","article-title":"Association of atrial arrhythmia and sinus node dysfunction in patients with catecholaminergic polymorphic ventricular tachycardia","volume":"71","author":"Sumitomo","year":"2007","journal-title":"Circ J"},{"key":"R4-20241204","doi-asserted-by":"crossref","first-page":"2124","DOI":"10.1253\/circj.CJ-66-0186","article-title":"Genetic background of catecholaminergic polymorphic ventricular tachycardia in Japan","volume":"84","author":"Kawamura","year":"2020","journal-title":"Circ J"},{"key":"R5-20241204","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1136\/heartjnl-2016-310509","article-title":"Nationwide experience of catecholaminergic polymorphic ventricular tachycardia caused by RyR2 mutations","volume":"103","author":"Broendberg","year":"2017","journal-title":"Heart"},{"key":"R6-20241204","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":"R7-20241204","doi-asserted-by":"crossref","first-page":"903","DOI":"10.3390\/jcm9040903","article-title":"Long-term follow-up of patients with catecholaminergic polymorphic ventricular arrhythmia","volume":"9","author":"Veith","year":"2020","journal-title":"J Clin Med"},{"key":"R8-20241204","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":"R9-20241204","doi-asserted-by":"crossref","first-page":"3997","DOI":"10.1093\/eurheartj\/ehac262","article-title":"2022 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death","volume":"43","author":"Zeppenfeld","year":"2022","journal-title":"Eur Heart J"},{"key":"R10-20241204","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1001\/jamacardio.2017.1320","article-title":"Efficacy of flecainide in the treatment of catecholaminergic polymorphic ventricular tachycardia: A randomized clinical trial","volume":"2","author":"Kannankeril","year":"2017","journal-title":"JAMA Cardiol"},{"key":"R11-20241204","doi-asserted-by":"crossref","first-page":"45","DOI":"10.15420\/aer.2016.3.3","article-title":"The role of flecainide in the management of catecholaminergic polymorphic ventricular tachycardia","volume":"5","author":"Lieve","year":"2016","journal-title":"Arrhythm Electrophysiol Rev"},{"key":"R12-20241204","doi-asserted-by":"crossref","first-page":"1825","DOI":"10.1016\/j.hrthm.2021.07.061","article-title":"Evaluation of age at symptom onset, proband status, and sex as predictors of disease severity in pediatric catecholaminergic polymorphic ventricular tachycardia","volume":"18","author":"Kallas","year":"2021","journal-title":"Heart Rhythm"},{"key":"R13-20241204","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/j.hrcr.2018.04.003","article-title":"A 35-year effective treatment of catecholaminergic polymorphic ventricular tachycardia with propafenone","volume":"5","author":"Marx","year":"2019","journal-title":"HeartRhythm Case Rep"},{"key":"R14-20241204","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1002\/mgg3.323","article-title":"Compound heterozygous CASQ2 mutations and long-term course of catecholaminergic polymorphic ventricular tachycardia","volume":"5","author":"Josephs","year":"2017","journal-title":"Mol Genet Genomic Med"},{"key":"R15-20241204","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1253\/circj.CJ-19-0720","article-title":"Co-phenotype of left ventricular non-compaction cardiomyopathy and atypical catecholaminergic polymorphic ventricular tachycardia in association with R169Q, a ryanodine receptor type 2 missense mutation","volume":"84","author":"Nozaki","year":"2020","journal-title":"Circ J"},{"key":"R16-20241204","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1161\/CIRCEP.114.002217","article-title":"Catecholaminergic polymorphic ventricular tachycardia in children: Analysis of therapeutic strategies and outcomes from an international multicenter registry","volume":"8","author":"Roston","year":"2015","journal-title":"Circ Arrhythm Electrophysiol"},{"key":"R17-20241204","doi-asserted-by":"crossref","first-page":"569","DOI":"10.2459\/JCM.0000000000000814","article-title":"The challenge of implantable cardioverter-defibrillator programming and shock interpretation in treatment-refractory catecholaminergic polymorphic ventricular tachycardia","volume":"20","author":"Roston","year":"2019","journal-title":"J Cardiovasc Med (Hagerstown)"}],"container-title":["Annals of Pediatric Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_101_24","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T20:03:08Z","timestamp":1787774588000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_101_24"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5]]},"references-count":17,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024]]}},"URL":"https:\/\/doi.org\/10.4103\/apc.apc_101_24","relation":{},"ISSN":["0974-2069","0974-5149"],"issn-type":[{"value":"0974-2069","type":"print"},{"value":"0974-5149","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5]]},"assertion":[{"value":"2024-05-19","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-08-27","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}