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Polymorphic catecholergic ventricular tachycardia

Condition where patients present with SYNCOPE and distinct stress-induced TACHYCARDIA without a prolonged QT-interval but heart is structurally normal. Onset occurs in childhood or adolescence and. Germline mutations in the RYR2 gene have been identified. OMIM: 180902
Also Known As:
Bidirectional Tachycardia Induced By Catecholamines; Catecholamine-induced polymorphic ventricular tachycardia; Catecholaminergic polymorphic ventricular tachycardia; Cpvt2; Familial polymorphic ventricular tachycardia; Stress-induced polymorphic ventricular tachycardia; Ventricular Tachycardia, Catecholaminergic Polymorphic, 1; Ventricular Tachycardia, Catecholaminergic Polymorphic, 2; Ventricular Tachycardia, Familial; Ventricular Tachycardia, Familial Polymorphic
Networked: 524 relevant articles (20 outcomes, 23 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Long QT Syndrome
2. Myocardial Ischemia (Ischemic Heart Diseases)
3. Cardiac Arrhythmias (Arrythmia)
4. Polymorphic catecholergic ventricular tachycardia
5. Cardiac Sudden Death (Sudden Cardiac Arrest)

Experts

1. Priori, Silvia G: 38 articles (10/2022 - 07/2002)
2. Ackerman, Michael J: 27 articles (01/2022 - 11/2004)
3. Napolitano, Carlo: 26 articles (01/2021 - 07/2002)
4. Wilde, Arthur A M: 22 articles (01/2022 - 10/2002)
5. Knollmann, Björn C: 20 articles (10/2022 - 04/2009)
6. Horie, Minoru: 19 articles (12/2022 - 02/2012)
7. Ohno, Seiko: 16 articles (12/2022 - 11/2014)
8. Chen, S R Wayne: 15 articles (01/2021 - 07/2010)
9. Swan, Heikki: 15 articles (01/2020 - 02/2002)
10. Tester, David J: 15 articles (01/2020 - 11/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Polymorphic catecholergic ventricular tachycardia:
1. Flecainide (Tambocor)FDA LinkGeneric
2. Ryanodine Receptor Calcium Release Channel (Ryanodine Receptor)IBA
3. IvabradineIBA
4. CalsequestrinIBA
5. CalciumIBA
01/01/2023 - "For example, the recently suggested role of Kir2.1 as a countercurrent to sarcoplasmic calcium reuptake might explain the arrhythmogenic mechanisms of ATS and its overlap with catecholaminergic polymorphic ventricular tachycardia. "
01/01/2022 - "Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by an arrhythmogenic mechanism involving disruption of calcium handling. "
01/01/2022 - "Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an exercise and emotional stress-induced life-threatening inherited heart rhythm disorder, characterized by an abnormal cellular calcium homeostasis. "
01/01/2018 - "We demonstrate the method by showing the contraction-calcium relation and measuring the transient time intervals in catecholaminergic polymorphic ventricular tachycardia patient specific iPSC-CMs and healthy controls. "
12/01/2016 - "A third patient from a consanguineous Sudanese family diagnosed with catecholaminergic polymorphic ventricular tachycardia (CPVT) had a homozygous splice site mutation (c.331+1G>A) in TECRL Analysis of intracellular calcium ([Ca2+]i) dynamics in human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) generated from this individual (TECRLHom-hiPSCs), his heterozygous but clinically asymptomatic father (TECRLHet-hiPSCs), and a healthy individual (CTRL-hiPSCs) from the same Sudanese family, revealed smaller [Ca2+]i transient amplitudes as well as elevated diastolic [Ca2+]i in TECRLHom-hiPSC-CMs compared with CTRL-hiPSC-CMs. "
6. Verapamil (Calan)FDA LinkGeneric
7. Calmodulin (Calcium-Dependent Activator Protein)IBA
8. Ion Channels (Ion Channel)IBA
9. CatecholaminesIBA
10. Anti-Arrhythmia AgentsIBA

Therapies and Procedures

1. Sympathectomy (Sympathectomies)
2. Therapeutics
3. Implantable Defibrillators (Implantable Cardioverter-Defibrillator)
4. Drug Therapy (Chemotherapy)
5. Defibrillators (Defibrillator)