| High prevalence of exercise-induced arrhythmias in catecholaminergic polymorphic ventricular tachycardia mutation-positive family members diagnosed by cascade genetic screening. | |
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MedLine Citation:
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PMID: 20106799 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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AIM: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited cardiac disease predisposing to life-threatening arrhythmias. We aimed to determine the prevalence of arrhythmias and efficacy of beta-blocker treatment in mutation-positive family members diagnosed by cascade genetic screening. METHODS AND RESULTS: Relatives of six unrelated CPVT patients were tested for the relevant mutation in the ryanodine receptor-2 gene. Mutation carriers underwent an exercise test at inclusion time and 3 months after the initiation of beta-blocker therapy in the highest tolerable dose. The occurrence of ventricular premature beats, couplets, and non-sustained ventricular arrhythmias (nsVT) were recorded in addition to the heart rate at which they occurred. Thirty family members were mutation carriers and were followed for 22 (13-288) months. Previous undiagnosed CPVT-related symptoms were reported by eight subjects. Exercise test induced ventricular arrhythmias in 23 of the 30 mutation carriers. On beta-blocker treatment, exercise-induced arrhythmias occurred at a lower heart rate (117 +/- 17 vs. 135 +/- 34 beats/min, P = 0.02) but at similar workload (P = 0.78). Beta-blocker treatment suppressed the occurrence of exercise-induced nsVT in three of the four patients, while less severe arrhythmias were unchanged. One patient died during follow-up. CONCLUSION: Exercise test revealed a high prevalence of arrhythmias in CPVT mutation carriers diagnosed by cascade genetic screening. beta-Blocker therapy appeared to suppress the most severe exercise-induced arrhythmias, while less severe arrhythmias occurred at a lower heart rate. |
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Authors:
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Kristina Hermann Haugaa; Ida Skrinde Leren; Knut Erik Berge; J?rn Bathen; Jan P?l Loennechen; Ole-Gunnar Anfinsen; Andreas Fr?h; Thor Edvardsen; Erik Kongsg?rd; Trond P Leren; Jan P Amlie |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2010-01-26 |
Journal Detail:
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Title: Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology Volume: 12 ISSN: 1532-2092 ISO Abbreviation: Europace Publication Date: 2010 Mar |
Date Detail:
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Created Date: 2010-02-22 Completed Date: 2010-05-07 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 100883649 Medline TA: Europace Country: England |
Other Details:
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Languages: eng Pagination: 417-23 Citation Subset: IM |
Affiliation:
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Department of Cardiology, Oslo University Hospital Rikshospitalet, Oslo 0027, Norway. kristina.haugaa@rr-research.no |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adrenergic beta-Antagonists / therapeutic use Adult Aged Catecholamines / genetics* Child Defibrillators, Implantable Electrocardiography Exercise* Exercise Test Family Health Female Genetic Testing Heart Rate Humans Male Middle Aged Point Mutation Prevalence Ryanodine Receptor Calcium Release Channel / genetics* Tachycardia, Ventricular / drug therapy, epidemiology*, genetics* Young Adult |
| Chemical | |
Reg. No./Substance:
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0/Adrenergic beta-Antagonists; 0/Catecholamines; 0/Ryanodine Receptor Calcium Release Channel |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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