Catecholaminergic Polymorphic Ventricular Tachycardia
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterised by adenergically mediated bidirectional and/or polymorphic ventricular tachycardia. CPVT is a significant cause of autopsy-negative sudden death in children and adolescents, although it can...
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| Format: | Article |
| Language: | English |
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Radcliffe Medical Media
2022-10-01
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| Series: | Arrhythmia & Electrophysiology Review |
| Online Access: | https://www.aerjournal.com/articleindex/aer.2022.09 |
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| author | Mohamed Abbas Chris Miles Elijah Behr |
| author_facet | Mohamed Abbas Chris Miles Elijah Behr |
| author_sort | Mohamed Abbas |
| collection | DOAJ |
| description | Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterised by adenergically mediated bidirectional and/or polymorphic ventricular tachycardia. CPVT is a significant cause of autopsy-negative sudden death in children and adolescents, although it can also affect adults. It is often caused by pathogenic variants in the cardiac ryanodine receptor gene as well as other rarer genes. Early identification and risk stratification is of major importance. β-blockers are the cornerstone of therapy. Sodium channel blockers, specifically flecainide, have an additive role. Left cardiac sympathetic denervation is playing an increasing role in suppression of arrhythmia and symptoms. Concerns have been raised, however, about the efficacy of implantable cardioverter defibrillator therapy and the risk of catecholamine driven proarrhythmic storms. In this review, we summarise the clinical characteristics, genetics, and diagnostic and therapeutic strategies for CPVT and describe recent advances and challenges. |
| format | Article |
| id | doaj-art-cdbe59c428e64c47a6b2a5c7af7aa516 |
| institution | OA Journals |
| issn | 2050-3369 2050-3377 |
| language | English |
| publishDate | 2022-10-01 |
| publisher | Radcliffe Medical Media |
| record_format | Article |
| series | Arrhythmia & Electrophysiology Review |
| spelling | doaj-art-cdbe59c428e64c47a6b2a5c7af7aa5162025-08-20T02:37:51ZengRadcliffe Medical MediaArrhythmia & Electrophysiology Review2050-33692050-33772022-10-011110.15420/aer.2022.09Catecholaminergic Polymorphic Ventricular TachycardiaMohamed Abbas0Chris Miles1Elijah Behr2Department of Cardiology, Royal Stoke University Hospital, Stoke-on-Trent, UKCardiovascular Clinical Academic Group, Molecular and Clinical Sciences Institute, St George’s, University of London and St George’s University Hospitals NHS Foundation Trust, London, UKCardiovascular Clinical Academic Group, Molecular and Clinical Sciences Institute, St George’s, University of London and St George’s University Hospitals NHS Foundation Trust, London, UKCatecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterised by adenergically mediated bidirectional and/or polymorphic ventricular tachycardia. CPVT is a significant cause of autopsy-negative sudden death in children and adolescents, although it can also affect adults. It is often caused by pathogenic variants in the cardiac ryanodine receptor gene as well as other rarer genes. Early identification and risk stratification is of major importance. β-blockers are the cornerstone of therapy. Sodium channel blockers, specifically flecainide, have an additive role. Left cardiac sympathetic denervation is playing an increasing role in suppression of arrhythmia and symptoms. Concerns have been raised, however, about the efficacy of implantable cardioverter defibrillator therapy and the risk of catecholamine driven proarrhythmic storms. In this review, we summarise the clinical characteristics, genetics, and diagnostic and therapeutic strategies for CPVT and describe recent advances and challenges.https://www.aerjournal.com/articleindex/aer.2022.09 |
| spellingShingle | Mohamed Abbas Chris Miles Elijah Behr Catecholaminergic Polymorphic Ventricular Tachycardia Arrhythmia & Electrophysiology Review |
| title | Catecholaminergic Polymorphic Ventricular Tachycardia |
| title_full | Catecholaminergic Polymorphic Ventricular Tachycardia |
| title_fullStr | Catecholaminergic Polymorphic Ventricular Tachycardia |
| title_full_unstemmed | Catecholaminergic Polymorphic Ventricular Tachycardia |
| title_short | Catecholaminergic Polymorphic Ventricular Tachycardia |
| title_sort | catecholaminergic polymorphic ventricular tachycardia |
| url | https://www.aerjournal.com/articleindex/aer.2022.09 |
| work_keys_str_mv | AT mohamedabbas catecholaminergicpolymorphicventriculartachycardia AT chrismiles catecholaminergicpolymorphicventriculartachycardia AT elijahbehr catecholaminergicpolymorphicventriculartachycardia |