Overview
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmogenic disorder characterized by ventricular tachycardia triggered by adrenergic stress, often linked to mutations in genes encoding ryanodine receptor type 2 (RyR2) and calsequestrin 2 (CASQ2). It poses significant risks of sudden cardiac death, particularly in young individuals. 1262566Diagnosis
Management
Special Populations
Key Recommendations
References
Showing 100 most recent of 190 indexed papers.
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Journal of psychosomatic research 2026. link 4 Moore OM, Sibrian-Vazquez M, Navarro-Garcia JA, Aguilar-Sanchez Y, Turkieltaub-Paredes MR, Lahiri SK et al.. Structure-activity optimization of ryanodine receptor modulators for the treatment of catecholaminergic polymorphic ventricular tachycardia. Heart rhythm 2025. link 5 Neves R, Bains S, Bos JM, van der Werf C, Bergeman AT, Peltenburg P et al.. International Multicenter Cohort Study on Beta-Blocker-Free Treatment Strategies for Catecholaminergic Polymorphic Ventricular Tachycardia Patients. JACC. Clinical electrophysiology 2025. link 6 Hsu GC, Wu MH, Chuang JY, Chiu SN, Lin MT, Lai LP et al.. Genetic and clinical characteristics of catecholaminergic polymorphic ventricular tachycardia in a Taiwanese nationwide cohort. Journal of the Formosan Medical Association = Taiwan yi zhi 2025. link 7 De Smet MAJ, De Becker B, François C, le Polain de Waroux JB, Knecht S, Duytschaever M et al.. Atrial Tachyarrhythmias With Ultra-Rapid Ventricular Response and Sudden Death in Patients Without Structural Heart Disease. JACC. Clinical electrophysiology 2025. link 8 Fitzgerald N, Lawley C, Morrish A, Tarca A, Marcondes L, Asakai H et al.. Catecholaminergic polymorphic ventricular tachycardia in children-incidence and trends in detection, presentation and management. Archives of disease in childhood 2025. link 9 Ranpura GN, Holliday M, Li S, Ross SB, Singer ES, Fraser ST et al.. Generation of an isogenic CRISPR/Cas9-corrected control induced pluripotent stem cell line from a patient with autosomal dominant catecholaminergic polymorphic ventricular tachycardia with a heterozygous variant in cardiac calsequestrin-2. Stem cell research 2025. link 10 Bachani N, Shah H, Bansal R, Soorampally V, Panicker GK, Lokhandwala Y. Characterization of Myocardial Recovery in Patients With Tachycardiomyopathy. Pacing and clinical electrophysiology : PACE 2025. link 11 Grogan A, Perelli RM, Ahn S, Jiang H, Jyothidasan A, Sood D et al.. A positive allosteric modulator of the β1AR with antagonist activity for catecholaminergic polymorphic ventricular tachycardia. The Journal of clinical investigation 2025. link 12 Askarinejad A, Esmaeili S, Dalili M, Biglari A, Kohansal E, Maleki M et al.. Catecholaminergic polymorphic ventricular tachycardia (and seizure) caused by a novel homozygous likely pathogenic variant in CASQ2 gene. Gene 2024. link 13 Xu B, Yang J, Liu F, Lv T, Li K, Yuan Y et al.. Clinical and genetic characteristics of catecholaminergic polymorphic ventricular tachycardia combined with left ventricular non-compaction. Cardiology in the young 2024. link 14 Rao SJ, Kanwal A, Kanwal A, Danilov A, Frishman WH. Landiolol: An Ultra-Short-Acting β-Blocker. Cardiology in review 2024. link 15 Renwick CE, Makhecha S, Till J. Case series: propranolol liquid in the treatment of tachyarrhythmias in neonates and infants: potential for errors. Cardiology in the young 2023. link 16 Zhang X, Li W, Lin A, Zou F, Marazzato J, Varrias D et al.. Outcomes of catheter ablation of atrial tachyarrhythmia guided exclusively by activation mapping. Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing 2023. link 17 DeCoy M, Page-Goertz C, Nofziger R, Besunder J, Raimer P, Gothard D et al.. Hemodynamic profile effects of PM101 amiodarone formulation in patients with post-operative tachyarrhythmias. Cardiology in the young 2023. link 18 Peltenburg PJ, Kallas D, Bos JM, Lieve KVV, Franciosi S, Roston TM et al.. An International Multicenter Cohort Study on β-Blockers for the Treatment of Symptomatic Children With Catecholaminergic Polymorphic Ventricular Tachycardia. Circulation 2022. link 19 Tunca Sahin G, Beattie RB, Uzun O. Favourable outcome for hydrops or cardiac failure associated with fetal tachyarrhythmia: a 20-year review. Cardiology in the young 2022. link 20 Bannister ML, MacLeod KT, George CH. Moving in the right direction: elucidating the mechanisms of interaction between flecainide and the cardiac ryanodine receptor. British journal of pharmacology 2022. link 21 Kryshtal DO, Blackwell DJ, Egly CL, Smith AN, Batiste SM, Johnston JN et al.. RYR2 Channel Inhibition Is the Principal Mechanism of Flecainide Action in CPVT. Circulation research 2021. link 22 Jimenez E, Cortez D, McGill M, Ambrose M. Peripartum management of a patient with catecholaminergic polymorphic ventricular tachycardia. Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc 2021. link 23 Pflaumer A, Wilde AAM, Charafeddine F, Davis AM. 50 Years of Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) - Time to Explore the Dark Side of the Moon. Heart, lung & circulation 2020. link 24 Pölönen RP, Swan H, Aalto-Setälä K. Mutation-specific differences in arrhythmias and drug responses in CPVT patients: simultaneous patch clamp and video imaging of iPSC derived cardiomyocytes. Molecular biology reports 2020. link 25 Cacheux M, Fauconnier J, Thireau J, Osseni A, Brocard J, Roux-Buisson N et al.. Interplay between Triadin and Calsequestrin in the Pathogenesis of CPVT in the Mouse. Molecular therapy : the journal of the American Society of Gene Therapy 2020. link 26 Wafae BG, da Silva RMF, Veloso HH. Propofol for sedation for direct current cardioversion. Annals of cardiac anaesthesia 2019. link 27 Cheung CC, Lieve KV, Roston TM, van der Ree MH, Deyell MW, Andrade JG et al.. Pregnancy in Catecholaminergic Polymorphic Ventricular Tachycardia. JACC. Clinical electrophysiology 2019. link 28 Tester DJ, Ackerman JP, Giudicessi JR, Ackerman NC, Cerrone M, Delmar M et al.. Plakophilin-2 Truncation Variants in Patients Clinically Diagnosed With Catecholaminergic Polymorphic Ventricular Tachycardia and Decedents With Exercise-Associated Autopsy Negative Sudden Unexplained Death in the Young. JACC. Clinical electrophysiology 2019. link 29 Lieve KVV, Verhagen JMA, Wei J, Bos JM, van der Werf C, Rosés I Noguer F et al.. Linking the heart and the brain: Neurodevelopmental disorders in patients with catecholaminergic polymorphic ventricular tachycardia. Heart rhythm 2019. link 30 Dallet C, Roney C, Martin R, Kitamura T, Puyo S, Duchateau J et al.. Cardiac Propagation Pattern Mapping With Vector Field for Helping Tachyarrhythmias Diagnosis With Clinical Tridimensional Electro-Anatomical Mapping Tools. IEEE transactions on bio-medical engineering 2019. link 31 Wangüemert Pérez F, Hernández Afonso JS, Groba Marco MDV, Caballero Dorta E, Álvarez Acosta L, Campuzano Larrea O et al.. Flecainide Reduces Ventricular Arrhythmias in Patients With Genotype RyR2-positive Catecholaminergic Polymorphic Ventricular Tachycardia. Revista espanola de cardiologia (English ed.) 2018. link 32 Richardson E, Spinks C, Davis A, Turner C, Atherton J, McGaughran J et al.. Psychosocial Implications of Living with Catecholaminergic Polymorphic Ventricular Tachycardia in Adulthood. Journal of genetic counseling 2018. link 33 Ozaki N, Nakamura Y, Suzuki T, Yoshimoto J, Toyohara K, Fukuhara H et al.. Safety and Efficacy of Radiofrequency Catheter Ablation for Tachyarrhythmia in Children Weighing Less Than 10 kg. Pediatric cardiology 2018. link 34 Klipp RC, Li N, Wang Q, Word TA, Sibrian-Vazquez M, Strongin RM et al.. EL20, a potent antiarrhythmic compound, selectively inhibits calmodulin-deficient ryanodine receptor type 2. Heart rhythm 2018. link 35 Pérez-Riera AR, Barbosa-Barros R, de Rezende Barbosa MPC, Daminello-Raimundo R, de Lucca AA, de Abreu LC. Catecholaminergic polymorphic ventricular tachycardia, an update. Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc 2018. link 36 Ho HT, Thambidorai S, Knollmann BC, Billman GE, Györke S, Kalyanasundaram A. Accentuated vagal antagonism paradoxically increases ryanodine receptor calcium leak in long-term exercised Calsequestrin2 knockout mice. Heart rhythm 2018. link 37 Syed YY. Landiolol: A Review in Tachyarrhythmias. Drugs 2018. link 38 Bosch C, Campuzano O, Sarquella-Brugada G, Cesar S, Perez-Serra A, Coll M et al.. A novel variant in RyR2 causes familiar catecholaminergic polymorphic ventricular tachycardia. Forensic science international 2017. link 39 Li N, Wang Q, Sibrian-Vazquez M, Klipp RC, Reynolds JO, Word TA et al.. Treatment of catecholaminergic polymorphic ventricular tachycardia in mice using novel RyR2-modifying drugs. International journal of cardiology 2017. link 40 Yang PC, Moreno JD, Miyake CY, Vaughn-Behrens SB, Jeng MT, Grandi E et al.. In silico prediction of drug therapy in catecholaminergic polymorphic ventricular tachycardia. The Journal of physiology 2016. link 41 Tülümen E, Schulze-Bahr E, Zumhagen S, Stallmeyer B, Seebohm G, Beckmann BM et al.. Early repolarization pattern: a marker of increased risk in patients with catecholaminergic polymorphic ventricular tachycardia. 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 2016. link 42 Zhang XH, Morad M. Calcium signaling in human stem cell-derived cardiomyocytes: Evidence from normal subjects and CPVT afflicted patients. Cell calcium 2016. link 43 Paavola J, Väänänen H, Larsson K, Penttinen K, Toivonen L, Kontula K et al.. Slowed depolarization and irregular repolarization in catecholaminergic polymorphic ventricular tachycardia: a study from cellular Ca2+ transients and action potentials to clinical monophasic action potentials and electrocardiography. 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 2016. link 44 Leren IS, Saberniak J, Majid E, Haland TF, Edvardsen T, Haugaa KH. Nadolol decreases the incidence and severity of ventricular arrhythmias during exercise stress testing compared with β1-selective β-blockers in patients with catecholaminergic polymorphic ventricular tachycardia. Heart rhythm 2016. link 45 Zhao YT, Huang YS, Yi Z. An 88-year-old man with syncope and an alternating axis. Heart (British Cardiac Society) 2016. link 46 Zhao YT, Valdivia CR, Gurrola GB, Powers PP, Willis BC, Moss RL et al.. Arrhythmogenesis in a catecholaminergic polymorphic ventricular tachycardia mutation that depresses ryanodine receptor function. Proceedings of the National Academy of Sciences of the United States of America 2015. link 47 Sánchez-Quintana D, Doblado-Calatrava M, Cabrera JA, Macías Y, Saremi F. Anatomical Basis for the Cardiac Interventional Electrophysiologist. BioMed research international 2015. link 48 Zhao YT, Valdivia CR, Gurrola GB, Hernández JJ, Valdivia HH. Arrhythmogenic mechanisms in ryanodine receptor channelopathies. Science China. Life sciences 2015. link 49 Arakawa J, Hamabe A, Aiba T, Nagai T, Yoshida M, Touya T et al.. A novel cardiac ryanodine receptor gene (RyR2) mutation in an athlete with aborted sudden cardiac death: a case of adult-onset catecholaminergic polymorphic ventricular tachycardia. Heart and vessels 2015. link 50 Cheung JW, Meli AC, Xie W, Mittal S, Reiken S, Wronska A et al.. Short-coupled polymorphic ventricular tachycardia at rest linked to a novel ryanodine receptor (RyR2) mutation: leaky RyR2 channels under non-stress conditions. International journal of cardiology 2015. link 51 Domingo D, Neco P, Fernández-Pons E, Zissimopoulos S, Molina P, Olagüe J et al.. Non-ventricular, Clinical, and Functional Features of the RyR2(R420Q) Mutation Causing Catecholaminergic Polymorphic Ventricular Tachycardia. Revista espanola de cardiologia (English ed.) 2015. link 52 Valle G, Boncompagni S, Sacchetto R, Protasi F, Volpe P. Post-natal heart adaptation in a knock-in mouse model of calsequestrin 2-linked recessive catecholaminergic polymorphic ventricular tachycardia. Experimental cell research 2014. link 53 Lawrenz W, Krogmann ON, Wieczorek M. 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. Cardiology in the young 2014. link 54 Banchs JE, Baquero GA, Nickolaus MJ, Wolbrette DL, Kelleman JJ, Samii S et al.. Clinical efficacy of dofetilide for the treatment of atrial tachyarrhythmias in adults with congenital heart disease. Congenital heart disease 2014. link 55 Burns KM, Evans F, Pearson GD, Berul CI, Kaltman JR. Rising charges and costs for pediatric catheter ablation. Journal of cardiovascular electrophysiology 2013. link 56 Watanabe H, van der Werf C, Roses-Noguer F, Adler A, Sumitomo N, Veltmann C et al.. Effects of flecainide on exercise-induced ventricular arrhythmias and recurrences in genotype-negative patients with catecholaminergic polymorphic ventricular tachycardia. Heart rhythm 2013. link 57 Al-Hassnan ZN, Tulbah S, Al-Manea W, Al-Fayyadh M. The phenotype of a CASQ2 mutation in a Saudi family with catecholaminergic polymorphic ventricular tachycardia. Pacing and clinical electrophysiology : PACE 2013. link 58 Loaiza R, Benkusky NA, Powers PP, Hacker T, Noujaim S, Ackerman MJ et al.. Heterogeneity of ryanodine receptor dysfunction in a mouse model of catecholaminergic polymorphic ventricular tachycardia. Circulation research 2013. link 59 King JH, Zhang Y, Lei M, Grace AA, Huang CL, Fraser JA. Atrial arrhythmia, triggering events and conduction abnormalities in isolated murine RyR2-P2328S hearts. Acta physiologica (Oxford, England) 2013. link 60 Zhang Y, Wu J, Jeevaratnam K, King JH, Guzadhur L, Ren X et al.. Conduction slowing contributes to spontaneous ventricular arrhythmias in intrinsically active murine RyR2-P2328S hearts. Journal of cardiovascular electrophysiology 2013. link 61 Lapage MJ, Bradley DJ, Dick M. Verapamil in infants: an exaggerated fear?. Pediatric cardiology 2013. link 62 Desai VC, Kelton CM, Czosek RJ, Heaton PC. Frequencies, costs, and complications of catheter ablation for tachyarrhythmias in children: 2000-2009. Pacing and clinical electrophysiology : PACE 2013. link 63 van der Werf C, Zwinderman AH, Wilde AA. Therapeutic approach for patients with catecholaminergic polymorphic ventricular tachycardia: state of the art and future developments. 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 2012. link 64 Kwong W, Neilson AL, Chiu CC, Gross GJ, Hamilton RM, Soucie L et al.. The effect of NavX on fluoroscopy times in pediatric catheter ablation. Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing 2012. link 65 Marjamaa A, Hiippala A, Arrhenius B, Lahtinen AM, Kontula K, Toivonen L et al.. Intravenous epinephrine infusion test in diagnosis of catecholaminergic polymorphic ventricular tachycardia. Journal of cardiovascular electrophysiology 2012. link 66 Pflaumer A, Davis AM. Guidelines for the diagnosis and management of Catecholaminergic Polymorphic Ventricular Tachycardia. Heart, lung & circulation 2012. link 67 Zhang Y, Fraser JA, Jeevaratnam K, Hao X, Hothi SS, Grace AA et al.. Acute atrial arrhythmogenicity and altered Ca(2+) homeostasis in murine RyR2-P2328S hearts. Cardiovascular research 2011. link 68 Alcalai R, Wakimoto H, Arad M, Planer D, Konno T, Wang L et al.. Prevention of ventricular arrhythmia and calcium dysregulation in a catecholaminergic polymorphic ventricular tachycardia mouse model carrying calsequestrin-2 mutation. Journal of cardiovascular electrophysiology 2011. link 69 Roux-Buisson N, Egéa G, Denjoy I, Guicheney P, Lunardi J. Germline and somatic mosaicism for a mutation of the ryanodine receptor type 2 gene: implication for genetic counselling and patient caring. 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 2011. link 70 Liu N, Ruan Y, Denegri M, Bachetti T, Li Y, Colombi B et al.. Calmodulin kinase II inhibition prevents arrhythmias in RyR2(R4496C+/-) mice with catecholaminergic polymorphic ventricular tachycardia. Journal of molecular and cellular cardiology 2011. link 71 Hilliard FA, Steele DS, Laver D, Yang Z, Le Marchand SJ, Chopra N et al.. Flecainide inhibits arrhythmogenic Ca2+ waves by open state block of ryanodine receptor Ca2+ release channels and reduction of Ca2+ spark mass. Journal of molecular and cellular cardiology 2010. link 72 Kalyanasundaram A, Bal NC, Franzini-Armstrong C, Knollmann BC, Periasamy M. The calsequestrin mutation CASQ2D307H does not affect protein stability and targeting to the junctional sarcoplasmic reticulum but compromises its dynamic regulation of calcium buffering. The Journal of biological chemistry 2010. link 73 Kashimura T, Briston SJ, Trafford AW, Napolitano C, Priori SG, Eisner DA et al.. In the RyR2(R4496C) mouse model of CPVT, β-adrenergic stimulation induces Ca waves by increasing SR Ca content and not by decreasing the threshold for Ca waves. Circulation research 2010. link 74 Fagundes A, DE Magalhaes LP, Russo M, Xavier E. Pharmacological treatment of electrical storm in cathecolaminergic polymorphic ventricular tachycardia. Pacing and clinical electrophysiology : PACE 2010. link 75 Celiker A, Erdoğan I, Karagöz T, Ozer S. Clinical experiences of patients with catecholaminergic polymorphic ventricular tachycardia. Cardiology in the young 2009. link 76 Györke S. Molecular basis of catecholaminergic polymorphic ventricular tachycardia. Heart rhythm 2009. link 77 Liu N, Rizzi N, Boveri L, Priori SG. Ryanodine receptor and calsequestrin in arrhythmogenesis: what we have learnt from genetic diseases and transgenic mice. Journal of molecular and cellular cardiology 2009. link 78 Fernández-Velasco M, Rueda A, Rizzi N, Benitah JP, Colombi B, Napolitano C et al.. Increased Ca2+ sensitivity of the ryanodine receptor mutant RyR2R4496C underlies catecholaminergic polymorphic ventricular tachycardia. Circulation research 2009. link 79 Katz G, Arad M, Eldar M. Catecholaminergic polymorphic ventricular tachycardia from bedside to bench and beyond. 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Ryanodine receptors and ventricular arrhythmias: emerging trends in mutations, mechanisms and therapies. Journal of molecular and cellular cardiology 2007. link 85 Postma AV, Denjoy I, Kamblock J, Alders M, Lupoglazoff JM, Vaksmann G et al.. Catecholaminergic polymorphic ventricular tachycardia: RYR2 mutations, bradycardia, and follow up of the patients. Journal of medical genetics 2005. link 86 Saul JP, Scott WA, Brown S, Marantz P, Acevedo V, Etheridge SP et al.. Intravenous amiodarone for incessant tachyarrhythmias in children: a randomized, double-blind, antiarrhythmic drug trial. Circulation 2005. link 87 Mayr AJ, Dünser MW, Ritsch N, Pajk W, Friesenecker B, Knotzer H et al.. High-dosage continuous amiodarone therapy to treat new-onset supraventricular tachyarrhythmias in surgical intensive care patients: an observational study. Wiener klinische Wochenschrift 2004. link 88 Ormaetxe JM, Sáez R, Arkotxa MF, Martinez-Alday JD. Catecholaminergic polymorphic ventricular tachycardia detected by an insertable loop recorder in a pediatric patient with exercise syncopal episodes. Pediatric cardiology 2004. link 89 Lin YH, Lai LP, Lin TK, Lin JL. Exercise-provoked bidirectional ventricular tachycardia in a young woman. Journal of the Formosan Medical Association = Taiwan yi zhi 2004. link 90 Kaup HJ, Hexamer M, Werner J. Morphological detection algorithms for the automatic implantable cardioverter/defibrillator (AICD). Biomedizinische Technik. Biomedical engineering 2004. link 91 Lokhandwala Y, Athawale S, Vora A. Tachyarrhythmias: the era of cure. Journal of the Indian Medical Association 2003. link 92 Khositseth A, Ramin KD, O'Leary PW, Porter CJ. Role of amiodarone in the treatment of fetal supraventricular tachyarrhythmias and hydrops fetalis. Pediatric cardiology 2003. link 93 Hirose M, Chiba S. Cellular mechanism of pituitary adenylate cyclase-activating polypeptide-induced atrial tachyarrhythmia in canine isolated arterially perfused right atria. Clinical and experimental pharmacology & physiology 2003. link 94 Fouron JC, Fournier A, Proulx F, Lamarche J, Bigras JL, Boutin C et al.. Management of fetal tachyarrhythmia based on superior vena cava/aorta Doppler flow recordings. Heart (British Cardiac Society) 2003. link 95 Laitinen PJ, Swan H, Kontula K. Molecular genetics of exercise-induced polymorphic ventricular tachycardia: identification of three novel cardiac ryanodine receptor mutations and two common calsequestrin 2 amino-acid polymorphisms. European journal of human genetics : EJHG 2003. link 96 George CH, Higgs GV, Lai FA. Ryanodine receptor mutations associated with stress-induced ventricular tachycardia mediate increased calcium release in stimulated cardiomyocytes. Circulation research 2003. link 97 Kron J. Clinical significance of device-related complications in clinical trials and implications for future trials: insights from the Antiarrhytmics Versus Implantable Defibrillators (AVID) trial. Cardiac electrophysiology review 2003. link 98 Bryant RM, Redfearn SP, Marangi D, Davenport JP, Kuntz ST, Schowengerdt KO. Catecholaminergic polymorphic ventricular tachycardia in a 3-year-old with occult myocarditis. Pacing and clinical electrophysiology : PACE 2002. link 99 Dornan RI. Anesthesia for a patient with catecholaminergic polymorphic ventricular tachycardia. Anesthesia and analgesia 2002. link 100 Postma AV, Denjoy I, Hoorntje TM, Lupoglazoff JM, Da Costa A, Sebillon P et al.. Absence of calsequestrin 2 causes severe forms of catecholaminergic polymorphic ventricular tachycardia. Circulation research 2002. link