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Bidirectional ventricular tachycardia: a hallmark of catecholaminergic polymorphic ventricular tachycardia.
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PMID:  22557844     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Catecholaminergic polymorphic ventricular tachycardia is a familial cardiac arrhythmia that is related to RYR2 or CASQ2 gene mutation. It occurs in patients with structurally normal heart and causes exercise-emotion triggered syncope and sudden cardiac death. We present a 13 year-old girl with recurrent episodes of exercise-related syncope and prior history of sudden death in a first degree relative.
Francisco Femenia; Raimundo Barbosa-Barros; Stela Vitorino Sampaio; Mauricio Arce; Andres Perez-Riera; Adrian Baranchuk
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Publication Detail:
Type:  Journal Article     Date:  2012-04-30
Journal Detail:
Title:  Indian pacing and electrophysiology journal     Volume:  12     ISSN:  0972-6292     ISO Abbreviation:  Indian Pacing Electrophysiol J     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-05-04     Completed Date:  2012-08-23     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  101157207     Medline TA:  Indian Pacing Electrophysiol J     Country:  India    
Other Details:
Languages:  eng     Pagination:  65-8     Citation Subset:  -    
Arrhythmia Unit. Cardiology Department. Hospital Espanol de Mendoza. Argentina.
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Journal Information
Journal ID (nlm-ta): Indian Pacing Electrophysiol J
Journal ID (iso-abbrev): Indian Pacing Electrophysiol J
Journal ID (publisher-id): Indian Pacing Electrophysiol J
ISSN: 0972-6292
Publisher: Indian Heart Rhythm Society
Article Information
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Copyright: © 2012 Femenia et al.
collection publication date: Season: Mar-Apr Year: 2012
Electronic publication date: Day: 30 Month: 4 Year: 2012
Volume: 12 Issue: 2
First Page: 65 Last Page: 68
ID: 3337370
PubMed Id: 22557844
Publisher Id: ipej120065-00

Bidirectional Ventricular Tachycardia: A Hallmark of Catecholaminergic Polymorphic Ventricular Tachycardia
Francisco Femenia1
Raimundo Barbosa-Barros2
Stela Vitorino Sampaio2
Mauricio Arce1
Andres Perez-Riera3
Adrian Baranchuk4
1Arrhythmia Unit. Cardiology Department. Hospital Espanol de Mendoza. Argentina
2Coronary Center. Hospital de Messejana "Dr. Carlos Alberto Studart Gomes". Fortaleza, Ceara. Brazil
3Faculdade de Medicina do ABC. Fundacao do ABC. Santo Andre, Sao Paulo. Brazil
4Heart Rhythm Service. Queen's University. Kingston, Ontario. Canada
Correspondence: Address for correspondence: Francisco Femenia. Av. San Martin 965. Godoy Cruz. Mendoza.

A 13-year-old girl with no cardiovascular history presented for evaluation of recurrent episodes of exercise-related syncope and prior history of sudden death in a first degree relative. The 12- lead electrocardiogram (ECG) at rest was normal and an echocardiogram confirmed a structurally normal heart. A 24-hour Holter monitoring (Figure 1, left panel) during physical activity showed the classic electrocardiographic manifestation of catecholaminergic polymorphic ventricular tachycardia (CPVT): bidirectional ventricular tachycardia (BVT), two alternating QRS complexes morphologies with different polarity (X and Y, Figure 1 right panel) with an XY interval of 240 ms and YX interval of 280 ms. It may be necessary to conduct a 12-lead ECG to confirm if those polarities represented right bundle branch block and left bundle branch block respectively. The BVT presented regular X-X and Y-Y intervals both at 520 ms. According to the classification originally proposed by Scherf and Kisch, the BVT presented here is a Type II BVT, defined by a regular and fixed alternation of short and long intervals.[1,2]

This tachycardia suddenly degenerated into self-limiting polymorphic ventricular tachycardia and ventricular fibrillation (Figure 1, left panel), and normal sinus rhythm resumed upon spontaneous cessation of ventricular fibrillation. The resting 12-lead ECG was normal after the event. (Figure 2)

The patient was treated with propanolol 120 mg/day. During a control exercise stress test the patient presented again with CPVT symptomatic by syncope. An implantable automatic cardioverter defibrillator was implanted (PRIZM DR, Guidant, MN, USA). Genetic screening was not performed at the time of writing this report.

During a ten year follow-up, the patient presented with three episodes of polymorphic ventricular tachycardia requiring ICD shocks. Propranolol dose was increased (160 mg/day) and the patient remained asymptomatic for the last two years.

CPVT is a rare but highly malignant (syncope or sudden cardiac death) genetic disease related to the mutation of cardiac ryanodine receptor gene (RYR2) or calsequestrin 2 gene (CASQ2), leading to an increase in intracellular Ca++ concentration, resulting in arrhythmia due to a cascade of delayed after depolarization and triggered activity [3-6], and the four distinguishing features of CPVT have subsequently been described by Coumel et al [7]: 1) normal resting electrocardiogram; 2) exercise- or emotion-induced severe ventricular tachycardia; 3) a typical pattern of bidirectional ventricular tachycardia; and 4) a structurally normal heart.

BVT has been described in a variety of clinical settings including digitalis toxicity, herbal aconite poisoning, hypokalemic periodic paralysis, myocarditis, coronary artery disease, metastatic cardiac tumors, non-ischemic dilated cardiomyopathy, Anderson-Tawil syndrome, and at the same time, has been recognized as a hallmark of CPVT.[8] It is usually triggered by exercise or emotional stress. It can be precipitated by premature ventricular contractions or ventricular bigeminy. As in this case, it can degenerate into polymorphic ventricular tachycardia and ventricular fibrillation.

The treatment is directed to suppress adrenergic activity, therefore beta-blockers are the most important drug in the treatment of CPVT. Beta-blockers are effective for the acute phase and maintenance treatment. However, if symptoms recur, ICD should be considered. Flecainide is also part of the armamentarium to treat this infrequent disease [9].

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Ylanen K,et al. Catecholaminergic polymorphic ventricular tachycardia Eur J Pediatr Year: 201016953520143088
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Baher AA,et al. Bidirectional ventricular tachycardia: ping pong in the His-Purkinje system Heart Rhythm Year: 2011859921118730
Coumel P,et al. Catecholaminergic-induced severe ventricular arrhythmias with Adams-Stokes syndrome in children: report of four cases Br Heart J Year: 19784028
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Biernacka EK,et al. Efficacy of flecainide in a patient with catecholaminergic polymorphic ventricular tachycardia Europace Year: 20111312920798117

Article Categories:
  • Case Report

Keywords: bidirectional tachycardia, ventricular tachycardia, catecholaminergic polymorphic ventricular tachycardia, syncope, sudden cardiac death.

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