Sudden cardiac death in Andersen Tawil syndrome
|
|
- Jayson Ryan
- 5 years ago
- Views:
Transcription
1 Europace (2007) 9, doi: /europace/eul188 CASE REPORT Sudden cardiac death in Andersen Tawil syndrome Stefan Peters 1 *, Eric Schulze-Bahr 2, Susan P. Etheridge 3, and Martin Tristani-Firouzi 3,4 1 Klinikum Dorothea Christiane Erxleben Quedlinburg, Academic Teaching Hospital of the University Hospital Magdeburg, Innere Medizin II-Kardiologie, Ditfurter Weg 24, Quedlinburg, Germany; 2 Genetics of Arrhythmias, Molecular Cardiology Section, Institute for Arteriosclerosis Research, University of Münster, Münster, Germany; 3 Department of Pediatrics, Division of Cardiology, University of Utah School of Medicine, Salt Lake City, UT, USA; and 4 Nora Eccles Harrision Cardiovascular Research and Training Institute, University of Utah School of Medicine, Salt Lake City, UT, USA Received 14 September 2006; accepted after revision 19 November 2006; online publish-ahead-of-print 1 February 2007 KEYWORDS Cardiac arrest; Bidirectional ventricular tachycardia; Polymorphic ventricular tachycardia; Genetic disorder; KCNJ2 mutation-negative Andersen Tawil Syndrome (ATS) is a heterogeneous autosomal dominant or sporadic disorder characterized by the clinical triad of periodic paralysis, dysmorphic features, and ventricular arrhythmias. 1,2 While mutations in KCNJ2 account for the majority of ATS cases, 35% of patients with the ATS phenotype are KCNJ2 mutation-negative. 1 Electrocardiographic manifestations of ATS include mild QTc prolongation, pronounced QUc prolongation, and prominent U-waves in KCNJ2 mutation-positive cases. 3,4 Ventricular ectopy is a common finding and includes frequent premature ventricular complexes (PVCs), bigeminy, polymorphic ventricular tachycardia (VT), and bidirectional VT. 1,3,5 Tachycardia burden is quite large in some ATS individuals, 6 with.50% of beats documented to be ventricular in origin. Despite a large tachycardia burden, most ATS patients are remarkably asymptomatic and sudden cardiac death is exceedingly rare. 1 Moreover, a family history of sudden cardiac death is rarely described. 5 Here, we describe the clinical characteristics of an individual with KCNJ2 mutation-negative ATS who presented with severe lifethreatening ventricular arrhythmias and ultimately expired despite ICD implantation. Case report A 48-year-old male with a past medical history of schizophrenia presented to a local emergency department with * Corresponding author. Tel: þ address: s.peters@klinikum-quedlinburg.de Andersen Tawil syndrome (ATS) is an autosomal dominant or sporadic disorder characterized by periodic paralysis, dysmorphic features, and ventricular arrhythmias. Although ventricular tachycardia burden is quite high sudden cardiac death in ATS is rare. We describe a case with sudden cardiac death due to electrical storm a few days after ICD implantation in KCNJ2 mutation-negative ATS. pre-syncopal symptoms and ECG documentation of spontaneous polymorphic ventricular ectopy and bidirectional VT at a rate of 135 bpm after development of periodic facial paralysis (Figure 1 A and B). After intravenous administration of 300 mg amiodarone, the patient developed rapid monomorphic VT (Figure 1C) associated with circulatory collapse. This rhythm degenerated into ventricular flutter that spontaneously converted into polymorphic VT. Ultimately, the polymorphic VT converted to bidirectional VT (Figure 1D) with stabilization of the patient s haemodynamic status. Subsequently, the bidirectional VT spontaneously reverted to sinus rhythm, and the patient was referred to our institution for further evaluation and management. The patient was receiving amisulprid 200 mg once daily for treatment of his schizophrenia. Standard ECG revealed QTc interval prolongation without T-wave abnormalities or prominent U-waves on this medication, measuring 520 ms (Figure 1E). Following discontinuation of amisulprid, the QTc shortened to ms (Figure 1F). Despite beta-blockade therapy, the patient continued to have frequent ventricular bigeminy, polymorphic VT, and bidirectional VT related to episodes of periodic facial paralysis. Family history of the patient was uneventful. Transthoracic echocardiography confirmed a structurally and functionally normal heart. Coronary angiography was normal. An ajmaline challenge was performed, but did not elicit coved ST segment elevation and right bundle branch block pattern in right precordial leads typical of Brugada syndrome. Bicycle stress test did not reveal & The European Society of Cardiology All rights reserved. For Permissions, please journals.permissions@oxfordjournals.org
2 SCD in Andersen Tawil syndrome 163 exercise-induced bidirectional VT, thus ruling out catecholaminergic polymorphic VT. Serum electrolyte levels were normal with and without episodes of periodic facial paralysis. The diagnosis of ATS was suggested by episodes of periodic paralysis of the facial musculature. Furthermore, the patient demonstrated evidence of mild dysmorphic features that included hypertelorism, thoracic scoliosis, and clinodactyly of the left fifth finger. Molecular genetic analysis was performed at the University Hospital of Münster, Germany. Direct sequencing of KCNJ2 revealed no evidence for any sequence variation. Furthermore, no mutations were detected in other genes known to cause LQT syndrome, including KCNQ1, KCNH2, and SCN5A. On the basis of the initial resuscitation and recurrent episodes of ventricular arrhythmias an ICD (Medtronic, Marquis DR, Minneapolis, MN, USA) was implanted. Programming included a VT zone at 171 bpm and ventricular fibrillation zone at 200 bpm. Within 8 days of ICD implantation, the patient developed recurrent syncope and 6 ICD discharges as an outpatient. He was evaluated at an outside emergency department, where he was noted to be haemodynamically stable despite polymorphic VT at a rate of 250 bpm (Figure 2). Without knowledge of his previous medical history, 200 mg intravenous amiodarone was administered which resulted in haemodynamic instability requiring cardiopulmonary resuscitation. The patient was transported to our institution, where he received multiple cardioversion attempts, intravenous magnesium boluses, and intravenous short acting beta-blockers. Following 3 h of resuscitation efforts, the patient ultimately expired while in a rhythm consistent with electromechanical dissociation. Stored electrograms of the ICD showed rapid polymorphic VT s with an RR interval of ms and short efficacy of each defibrillation shock with spontaneously recurrent VT. Discussion Andersen Tawil syndrome is a rare disorder and as such, relatively little is known regarding the natural history of cardiac disease. Ventricular ectopy is a common cardiac manifestion of ATS, including frequent PVCs, ventricular bigeminy, and non-sustained polymorphic and bidirectional VT. Although tachycardia burden may be large in some individuals with ATS, life-threatening arrhythmias are uncommon and sudden death is rare. 3,4 The individual reported here warrants careful review and consideration in light of his ultimate demise from uncontrollable ventricular arrhythmias. Mutations in KCNJ2 account for the majority of ATS cases. 5 KCNJ2 encodes the Kir2.1 channel which is the major component of the inward rectifier K þ current, I K1. A reduction in Kir2.1, caused by transfection of dominant-negative constructs, induces spontaneous electrical activity in normally quiescent ventricular myocyctes. 7,8 In silico reduction of I K1 causes prolongation of the most terminal portion of the cardiac action potential and induces spontaneous depolarizations triggered by forward mode of the Na þ /Ca 2þ exchanger. 3,9 The molecular basis for KCNJ2-mutation negative ATS is not known. However, KCNJ2 mutation-negative ATS patients are phenotypically indistinguishable from mutation-positive individuals with respect to periodic paralysis, cardiac arrhythmias, and skeletal dysmorphisms. 1 This suggests that mutation-negative patients harbour a mutation in a gene that ultimately results in reduced function of Kir2.1 or I K1 channels. Complicating this patient s history was the treatment of his schizophrenia with amisulpride. QTc prolongation in the setting of amisulpride toxicity and dose-dependent QTc prolongation, torsade de pointes, and lethal ventricular fibrillation 13 have been described. A genetic predisposition has long been proposed as a risk factor for acquired LQTS. 14 The current patient s predisposition to acquired LQTS was not due to a mutation or rare polymorphism in the common genes that cause inherited LQTS, as this was excluded by molecular genetic analysis. We cannot fully discount the possibility that he harboured a mutation or polymorphism in an as-of-yet unidentified LQTS gene (at most 5 10%). However, we propose that the predisposition to acquired LQTS was exacerbated by KCNJ2 Figure 1 (A) Polymorphic ventricular ectopy. (B) Bidirectional VT after facial paralysis. (C) Rapid monomorphic VT after 300 mg amiodarone. (D) Spontaneous conversion to bidirectional VT. (E) Standard ECG with QTc prolongation of 520 ms on amisulprid. (F) Standard ECG with a QTc interval of ms after discontinuation of amisulprid.
3 164 S. Peters et al. Figure 1 mutation-negative ATS. Indeed, simulations of ATS mutations in virtual ventricular tissue indicate that the contribution of I Kr to phase 3 repolarization increases substantially in the setting of reduced I K1. 15 Thus, ATS patients may be particularly susceptible to changes in I Kr given the baseline reduced repolarization reserve and the dependence upon I Kr as a substitute for reduced I K1. In addition to amisulpride, the patient received intravenous amiodarone, which is also reported to prolong QTc. However, unlike many QTc-prolonging agents, amiodarone is rarely torsadogenic primarily due to its propensity to block multiple channels resulting in reduced transmural dispersion of repolarization. 16 This may be less predictable in ATS patients with K þ channel dysfunction. We suggest that the pro-arrhythmic effect of amiodarone was related, in part, to an underlying reduced repolarization reserve as a consequence of ATS. It remains unclear, why the patient subsequently developed rapid polymorphic VT while off Continued. amisulprid that was refractory to internal and external cardioversion. The use of ICD secondary prevention in ATS population has not been systematically assessed, primarily due to the rarity of catastrophic events in this disease. The frequent yet asymptomatic VT typical of ATS evokes the question of optimal programming strategy. We believe that ICD implantation was indicated in this patient with syncope and documented VT. Ventricular tachycardia detection zone set at 171 bpm should have been sufficient to avoid repeated ICD discharges given that the rate of bidirectional VT was,150 bpm. Yet, the patient developed an electrical storm and was not stabilized (or worsened) by amiodarone and recurrent defibrillation attempts. In summary, ATS is a rare channelopathy that complicates our understanding of the natural history of ventricular arrhythmias. Although ATS subjects are predisposed to ventricular arrhythmias, these arrhythmias rarely degenerate
4 SCD in Andersen Tawil syndrome 165 Figure 2 Figure 1 Continued. Polymorphic VT at a rate of 250 bpm. into lethal ventricular fibrillation. The clinical course in this patient was likely affected by the use of pharmacological agents that prolong QTc. Although clearly part of the recommendations in patients with LQTS, the avoidance of agents that lengthen repolarization has not become a routine recommendation in ATS patients. In light of the lethal clinical course of the patient reported here, we recommend against the use of any QTc-prolonging medication and urge extreme caution in treating ventricular arrhythmias in patients with documented or suspected ATS. References 1. Venance SL, Cannon SC, Failho D, Fontaine B, Hanna MG, Ptacek LJ et al. The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain 2006;129: Plaster NM, Tawil R, Tristani-Firouzi M, Canun S, Bendahhou S, Tsunoda A et al. Mutations in Kir2.1 cause the developmental and episodic electrical phenotypes of Andersen s syndrome. Cell 2001;105: Tristani-Firouzi M, Jensen JL, Donaldson MR, Sansone V, Meola G, Hahn A et al. Functional and clinical characterization of KCNJ2 mutations associated with LQT7 (Andersen syndrome). J Clin Invest 2002;110:
5 166 S. Peters et al. 4. Zhang L, Benson DW, Tristani-Firouzi M, Ptacek LJ, Schwartz PJ, George AL et al. Electrocardiographic features in Andersen Tawil syndrome patients with KCNJ2 mutations: characteristic T-U-wave patterns predict the KCNJ2 genotype. Circulation 2005;111: Donaldson MR, Jensen JL, Tristani-Firouzi M, Tawil R, Bendahhou S, Suarez WA et al. PIP(2) binding residues of Kir2.1 are common targets of mutations causing Andersen syndrome. Neurology 2003;60: Chun TU, Epstein MR, Dick M II, Andelfinger G, Ballester L, Vanoye VG et al. Polymorphic ventricular tachycardia and KCNJ2 mutations. Heart Rhythm 2004;1: Miake J, Marban E, Nuss HB. Biological pacemaker created by gene transfer. Nature 2002;419: Lange PS, Er F, Gassanov N, Hoppe UC. Andersen mutations of KCNJ2 suppress the native inward rectifier current IK1 in a dominant-negative fashion. Cardiovasc Res 2003;59: Silva J, Rudy Y. Mechanism of pacemaking in I(K1)-downregulated myocytes. Circ Res 2003;92: Tracqui A, Mutter-Schmidt C, Kintz P, Berton C, Mangin P. Amisulpride poisoning: a report on two cases. Hum Exp Toxicol 1995;14: Stollberger C, Huber JO, Finsterer J. Antipsychotic drugs and QT prolongation. Int Clin Psychopharmacol 2005;20: Ward DI. Two cases of amisulpride overdose: a cause for prolonged QT syndrome. Emerg Med Australas 2005;17: Sugiyama A, Satoh Y, Shiina H, Takeda S, Hashimoto K. Torsadegenic action of the antipsychotic drug sulpiride assessed using in vivo canine models. J Cardiovasc Pharmacol 2002;40: Roden DM, George AL Jr. The genetic basis of variability in drug responses. Nat Rev Drug Discov 2002;1: Seemann G, Sachse FB, Weiss DL, Ptáček LP, Tristani-Firouzi M. Modeling of IK1 mutations in human left ventricular myocytes and tissue. Am J Physiol Heart Circ Physiol 2006 [Epub ahead of print]. 16. Fenichel RR, Malik M, Antzelevitch C, Sanguinett M, Roden DM, Priori SG et al. Drug-induced torsades de pointes and implications for drug development. J Cardiovasc Electrophysiol 2004;15:
Case Demonstrations in Congenital and Acquired Long QT Syndrome
Case Demonstrations in Congenital and Acquired Long QT Syndrome Can You Make A Correct ECG Interpretation? Li Zhang, MD; 1-2 G. Michael Vincent, MD 1 1. LQTS Studies, Department t of Medicine i LDS Hospital,
More informationBasics of Structure/Function of Sodium and Potassium Channels Barry London, MD PhD
Basics of Structure/Function of Sodium and Potassium Channels Barry London, MD PhD University of Pittsburgh Medical Center Pittsburgh, PA International Symposium of Inherited Arrhythmia Disorders and Hypertrophic
More informationPearls of the ESC/ERS Guidelines 2015 Channelopathies
Pearls of the ESC/ERS Guidelines 2015 Channelopathies Carina Blomstrom Lundqvist Dept Cardiology, Uppsala, Sweden Content 2015 ESC Guidelines for the Management of Patients with Ventricular Arrhythmias
More informationName of Presenter: Marwan Refaat, MD
NAAMA s 24 th International Medical Convention Medicine in the Next Decade: Challenges and Opportunities Beirut, Lebanon June 26 July 2, 2010 I have no actual or potential conflict of interest in relation
More informationCONGENITAL LONG QT SYNDROME(CLQTS) ASSOCIATED WITH COMPLETE ATRIOVENTRICULAR BLOCK. A CASE REPORT.
CONGENITAL LONG QT SYNDROME(CLQTS) ASSOCIATED WITH COMPLETE ATRIOVENTRICULAR BLOCK. A CASE REPORT. SAHA Annual Congress 2017. Samkelo Jiyana, Adele Greyling, Andile Nxele, ZM,Makrexeni,L.Pepeta. BACKGROUND
More informationThe Role of Defibrillator Therapy in Genetic Arrhythmia Syndromes
The Role of Defibrillator Therapy in Genetic Arrhythmia Syndromes RHEA C. PIMENTEL, MD, FACC, FHRS UNIVERSITY OF KANSAS HOSPITAL MID AMERICA CARDIOLOGY AUGUST 19, 2012 Monogenic Arrhythmia Syndromes Mendelian
More informationCME Article Brugada pattern masking anterior myocardial infarction
Electrocardiography Series Singapore Med J 2011; 52(9) : 647 CME Article Brugada pattern masking anterior myocardial infarction Seow S C, Omar A R, Hong E C T Cardiology Department, National University
More informationClinical and Electrocardiographic Characteristics of Patients with Brugada Syndrome: Report of Five Cases of Documented Ventricular Fibrillation
J Arrhythmia Vol 25 No 1 2009 Original Article Clinical and Electrocardiographic Characteristics of Patients with Brugada Syndrome: Report of Five Cases of Documented Ventricular Fibrillation Seiji Takashio
More informationDrugs Controlling Myocyte Excitability and Conduction at the AV node Singh and Vaughan-Williams Classification
Drugs Controlling Myocyte Excitability and Conduction at the AV node Singh and Vaughan-Williams Classification Class I Na Channel Blockers Flecainide Propafenone Class III K channel Blockers Dofetilide,
More informationΤΙ ΠΡΕΠΕΙ ΝΑ ΓΝΩΡΙΖΕΙ ΟΓΕΝΙΚΟΣ ΚΑΡΔΙΟΛΟΓΟΣ ΓΙΑ ΤΙΣ ΔΙΑΥΛΟΠΑΘΕΙΕΣ
ΤΙ ΠΡΕΠΕΙ ΝΑ ΓΝΩΡΙΖΕΙ ΟΓΕΝΙΚΟΣ ΚΑΡΔΙΟΛΟΓΟΣ ΓΙΑ ΤΙΣ ΔΙΑΥΛΟΠΑΘΕΙΕΣ ΣΤΕΛΙΟΣ ΠΑΡΑΣΚΕΥΑÏΔΗΣ ΔΙΕΥΘΥΝΤΗΣ ΕΣΥ Α Καρδιολογική Κλινική ΑΠΘ, Νοσοκομείο ΑΧΕΠΑ, Θεσσαλονίκη NO CONFLICT OF INTEREST Sudden Cardiac Death
More informationGenetic Testing for Cardiac Ion Channelopathies
Applies to all products administered or underwritten by Blue Cross and Blue Shield of Louisiana and its subsidiary, HMO Louisiana, Inc.(collectively referred to as the Company ), unless otherwise provided
More informationFANS Paediatric Pathway for Inherited Arrhythmias*
FANS Paediatric Pathway for Inherited Arrhythmias* The pathway is based on the HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes
More informationMedical Policy An Independent Licensee of the Blue Cross and Blue Shield Association
Genetic Testing for Page 1 of 23 Medical Policy An Independent Licensee of the Blue Cross and Blue Shield Association Title: Genetic Testing for Professional Institutional Original Effective Date: August
More informationICD in a young patient with syncope
ICD in a young patient with syncope Konstantinos P. Letsas, MD, FESC Second Department of Cardiology Evangelismos General Hospital of Athens Athens, Greece Case presentation A 17-year-old apparently healthy
More informationSyncope in patients with inherited arrhythmogenic syndromes. Is it enough to justify ICD implantation?
Innovations in Interventional Cardiology and Electrophysiology Thessaloniki 2014 Syncope in patients with inherited arrhythmogenic syndromes. Is it enough to justify ICD implantation? K. Letsas, MD, FESC
More informationThe pill-in-the-pocket strategy for paroxysmal atrial fibrillation
The pill-in-the-pocket strategy for paroxysmal atrial fibrillation KONSTANTINOS P. LETSAS, MD, FEHRA LABORATORY OF CARDIAC ELECTROPHYSIOLOGY EVANGELISMOS GENERAL HOSPITAL OF ATHENS ARRHYTHMIAS UPDATE,
More informationWINDLAND SMITH RICE SUDDEN DEATH GENOMICS LABORATORY
Learning Objectives to Disclose: To CRITIQUE the ICD and its role in the treatment of BrS, CPVT, and LQTS WINDLAND SMITH RICE SUDDEN DEATH GENOMICS LABORATORY Conflicts of Interest to Disclose: Consultant
More informationGenetic Testing for Cardiac Ion Channelopathies
Genetic Testing for Cardiac Ion Channelopathies Policy Number: 2.04.43 Last Review: 11/2018 Origination: 6/2007 Next Review: 11/2019 Policy Blue Cross and Blue Shield of Kansas City (Blue KC) will provide
More informationGenetics of Sudden Cardiac Death. Geoffrey Pitt Ion Channel Research Unit Duke University. Disclosures: Grant funding from Medtronic.
Genetics of Sudden Cardiac Death Geoffrey Pitt Ion Channel Research Unit Duke University Disclosures: Grant funding from Medtronic Duke U N I V E R S I T Y Sudden Cardiac Death High incidence 50-100 per
More informationLeft cardiac sympathectomy to manage beta-blocker resistant LQT patients
Left cardiac sympathectomy to manage beta-blocker resistant LQT patients Lexin Wang, M.D., Ph.D. Introduction Congenital long QT syndrome (LQTS) is a disorder of prolonged cardiac repolarization, manifested
More informationVentricular tachycardia Ventricular fibrillation and ICD
EKG Conference Ventricular tachycardia Ventricular fibrillation and ICD Samsung Medical Center CCU D.I. Hur Ji Won 2006.05.20 Ventricular tachyarrhythmia ventricular tachycardia ventricular fibrillation
More informationAre there low risk patients in Brugada syndrome?
Are there low risk patients in Brugada syndrome? Pedro Brugada MD, PhD Andrea Sarkozy MD Risk stratification in Brugada syndrome In the last years risk stratification in Brugada syndrome has become the
More informationCorporate Medical Policy
Corporate Medical Policy Genetic Testing for Cardiac Ion Channelopathies File Name: Origination: Last CAP Review: Next CAP Review: Last Review: genetic_testing_for_cardiac_ion_channelopathies 10/2008 4/2018
More informationNIH Public Access Author Manuscript Heart Rhythm. Author manuscript; available in PMC 2007 March 1.
NIH Public Access Author Manuscript Published in final edited form as: Heart Rhythm. 2006 March ; 3(3): 328 335. Cellular basis for electrocardiographic and arrhythmic manifestations of Andersen-Tawil
More informationQuinidine for Brugada syndrome: Panacea or poison?
Quinidine for Brugada syndrome: Panacea or poison? Jo-Jo Hai, MBBS, * Chun-Ka Wong, MBBS, * Pak-Hei Chan, MBBS, * Hung-Fat Tse, MD, PhD, * Tak-Cheung Yung, MBBS, Chung-Wah Siu, MD From the * Division of
More informationBrugada syndrome is a cardiac disease caused by an
Efficacy of Quinidine in High-Risk Patients With Brugada Syndrome Bernard Belhassen, MD; Aharon Glick, MD; Sami Viskin, MD Background Automatic implantable cardioverter-defibrillator therapy is considered
More informationLong Q. Long QT Syndrome. A Guide for
Long Q Long QT Syndrome A Guide for Introduction Long QT syndrome (LQTS) is a genetic heart disorder due to the malfunction of cardiac ion channels that results in 4,000 deaths annually in the United States
More informationSection: Effective Date: Subsection: Original Policy Date: Subject: Page: Last Review Status/Date: Background
Genetic Testing for Cardiac Ion Last Review Status/Date: March 2014 Genetic Testing for Cardiac Ion Description Page: 1 of 22 Genetic testing is available for patients suspected of having cardiac ion channelopathies
More informationSilvia G Priori MD PhD
The approach to the cardiac arrest survivor Silvia G Priori MD PhD Molecular Cardiology, IRCCS Fondazione Salvatore Maugeri Pavia, Italy AND Leon Charney Division of Cardiology, Cardiovascular Genetics
More informationTailored therapy in long QT syndrome
Tailored therapy in long QT syndrome Dominic Abrams St. Bartholomew s & Great Ormond Street Hospitals London, UK Disclosures None Tailored therapy in long QTS Which patients should have tailored therapy...?...
More informationRhythm and Blues Drugs and QT Prolongation
Rhythm and Blues Drugs and QT Prolongation Dr Martin Quinn St Vincents University Hospital Irish Medication Safety Network conference Farmleigh 18 Oct 2013 Drugs and QT Prolongation Anti-psychotic, antidepressant,
More informationIntraoperative and Postoperative Arrhythmias: Diagnosis and Treatment
Intraoperative and Postoperative Arrhythmias: Diagnosis and Treatment Karen L. Booth, MD, Lucile Packard Children s Hospital Arrhythmias are common after congenital heart surgery [1]. Postoperative electrolyte
More informationArrhythmias (II) Ventricular Arrhythmias. Disclosures
Arrhythmias (II) Ventricular Arrhythmias Amy Leigh Miller, MD, PhD Cardiovascular Electrophysiology, Brigham & Women s Hospital Disclosures None Rhythms and Mortality Implantable loop recorder post-mi
More informationJ Wave Syndromes. Osama Diab Lecturer of Cardiology Ain Shams University
J Wave Syndromes Osama Diab Lecturer of Cardiology Ain Shams University J Wave Syndromes Group of electric disorders characterized by > 1 mm elevation of the J point or prominent J wave with or without
More informationStefan Peters* Introduction. Methods. * Corresponding author. Tel: þ address:
Europace (2008) 10, 816 820 doi:10.1093/europace/eun030 Arrhythmogenic right ventricular dysplasia-cardiomyopathy and provocable coved-type ST-segment elevation in right precordial leads: clues from long-term
More informationProlonged QT Syndromes: Congenital and Acquired
Prolonged QT Syndromes: Congenital and Acquired April 30, 2014 Elizabeth S. Kaufman, MD I have no financial disclosures. MetroHealth Campus, Case Western Reserve University Prolonged QT Syndromes Congenital
More informationElectrophysiologic investigation in Brugada syndrome
European Heart Journal (2002) 23, 1394 1401 doi:10.1053/euhj.2002.3256, available online at http://www.idealibrary.com on Electrophysiologic investigation in Brugada syndrome Yield of programmed ventricular
More informationComparison of different proarrhythmia biomarkers in isolated rabbit hearts
Comparison of different proarrhythmia biomarkers in isolated rabbit hearts Summary of PhD Thesis Szabolcs Orosz, MSc Supervisor: Attila Farkas MD, PhD 2nd Dept. of Internal Medicine and Cardiology Centre
More informationLong QT Syndrome in Children in the Era of Implantable Defibrillators
Journal of the American College of Cardiology Vol. 50, No. 14, 2007 2007 by the American College of Cardiology Foundation ISSN 0735-1097/07/$32.00 Published by Elsevier Inc. doi:10.1016/j.jacc.2007.05.042
More informationThe patient with (without) an ICD and heart failure: Management of electrical storm
ISHNE Heart Failure Virtual Symposium April 2008 The patient with (without) an ICD and heart failure: Management of electrical storm Westfälische Wilhelms-Universität Münster Günter Breithardt, MD, FESC,
More informationExercise guidelines in athletes with isolated repolarisation abnormalities and structurally normal heart.
Exercise guidelines in athletes with isolated repolarisation abnormalities and structurally normal heart. Hanne Rasmusen Consultant cardiologist, PhD Dept. of Cardiology Bispebjerg University Hospital
More informationChapter 16: Arrhythmias and Conduction Disturbances
Complete the following. Chapter 16: Arrhythmias and Conduction Disturbances 1. Cardiac arrhythmias result from abnormal impulse, abnormal impulse, or both mechanisms together. 2. is the ability of certain
More informationAcquired QT prolongation and Torsades de Pointes: a review and case report
Acquired QT prolongation and Torsades de Pointes: a review and case report Alastair Gray, Conor McQuillan Royal Victoria Hospital Belfast Introduction As early as the 1920 s it was noted that several drugs
More informationP. Brugada 1, R. Brugada 2 and J. Brugada 3. Introduction. U.S.A.; 3 Unitat d Arritmias, Hospital Clinic, Barcelona, Spain
European Heart Journal (2000) 21, 321 326 Article No. euhj.1999.1751, available online at http://www.idealibrary.com on Sudden death in patients and relatives with the syndrome of right bundle branch block,
More informationSudden cardiac death: Primary and secondary prevention
Sudden cardiac death: Primary and secondary prevention By Kai Chi Chan Penultimate Year Medical Student St George s University of London at UNic Sheba Medical Centre Definition Sudden cardiac arrest (SCA)
More informationAsaad Khoury 2,3 MD, Monther Boulos 1,3 MD, Mahmoud Suleiman 1,3 MD, Miry Blich 1,3 MD, Michael Eldar 4 MD, Ibrahim Marai 1,3 MD,
Flecainide therapy suppresses exercise induced ventricular arrhythmias in patients with CASQ2 associated catecholaminergic polymorphic ventricular tachycardia Asaad Khoury 2,3 MD, Monther Boulos 1,3 MD,
More informationManagement and Treatment of Andersen-Tawil Syndrome (ATS)
Neurotherapeutics: The Journal of the American Society for Experimental NeuroTherapeutics Management and Treatment of Andersen-Tawil Syndrome (ATS) Valeria Sansone* and Rabi Tawil *Department of Neurology,
More informationFunctional and clinical characterization of KCNJ2 mutations associated with LQT7 (Andersen syndrome)
Functional and clinical characterization of KCNJ2 mutations associated with LQT7 (Andersen syndrome) Martin Tristani-Firouzi, 1 Judy L. Jensen, 2 Matthew R. Donaldson, 2 Valeria Sansone, 3 Giovanni Meola,
More informationWhen the rhythm of life is disturbed
Brugada Syndrome has the capacity to cause abnormal heart rhythms originating in the upper chambers of the heart When the rhythm of life is disturbed Cardiovascular disease continues to be a leading cause
More informationIs There a Genomic Basis to Acquired Channelopathic disease
Is There a Genomic Basis to Acquired Channelopathic disease Yaniv Bar-Cohen, M.D. Associate Professor of Pediatrics Division of Cardiology / Electrophysiology Children s Hospital Los Angeles Keck School
More informationΠαναγιώτης Ιωαννίδης. Διευθυντής Τμήματος Αρρυθμιών & Επεμβατικής Ηλεκτροφυσιολογίας Βιοκλινικής Αθηνών
Διαστρωμάτωση κινδύνου για αιφνίδιο καρδιακό θάνατο σε ασθενείς που δεν συμπεριλαμβάνονται σε μεγάλες κλινικές μελέτες «Ασθενείς με ηλεκτρικά νοσήματα» Παναγιώτης Ιωαννίδης Διευθυντής Τμήματος Αρρυθμιών
More informationVentricular arrhythmias
Ventricular arrhythmias Assoc.Prof. Lucie Riedlbauchová, MD, PhD Department of Cardiology University HospitalMotol and2nd FacultyofMedicine, Charles University in Prague Definition and classification Ventricular
More informationHow to manage a patient with short QT syndrome?
How to manage a patient with short QT syndrome? Torino, 27 ottobre2012 Carla Giustetto Division of Cardiology University of Torino QT 280 ms QTc 260 ms Narrow, tall and peaked T waves High incidence of
More informationShort QT syndrome: A case report and review of literature
Resuscitation (2006) 71, 115 121 CASE REPORT Short QT syndrome: A case report and review of literature Li Xiong Lu, Wei Zhou, Xingyu Zhang, Qin Cao, Kanglong Yu, Changqing Zhu Department of Emergency and
More informationSudden Cardiac Death What an electrophysiologist thinks a cardiologist should know
Sudden Cardiac Death What an electrophysiologist thinks a cardiologist should know Steven J. Kalbfleisch, M.D. Medical Director Electrophysiology Laboratory Ross Heart Hospital Wexner Medical Center Sudden
More informationAtrial fibrillation in Cardiac Channelopathies
Atrial fibrillation in Cardiac Channelopathies Thejus, Jayachandran Francis, Johnson Introduction Atrial fibrillation is the commonest arrhythmia encountered in day-to-day clinical practice. Its prevalence
More informationTachycardias II. Štěpán Havránek
Tachycardias II Štěpán Havránek Summary 1) Supraventricular (supraventricular rhythms) Atrial fibrillation and flutter Atrial ectopic tachycardia / extrabeats AV nodal reentrant a AV reentrant tachycardia
More informationthat number is extremely high. It s 16 episodes, or in other words, it s 14, one-four, ICD shocks per patient per day.
Doctor Karlsner, Doctor Schumosky, ladies and gentlemen. It s my real pleasure to participate in this session on controversial issues in the management of ventricular tachycardia and I m sure that will
More informationA case of Brugada syndrome coexisting with vasospastic angina: Caution should be taken when using calcium channel blockers
Journal of Cardiology Cases (2011) 4, e143 e147 Available online at www.sciencedirect.com jou rn al h om epa g e: www.elsevier.com/locate/jccase Case Report A case of Brugada syndrome coexisting with vasospastic
More informationMedical Policy An independent licensee of the Blue Cross Blue Shield Association
Genetic Testing for Page 1 of 29 Medical Policy An independent licensee of the Blue Cross Blue Shield Association Title: Genetic Testing for Professional Institutional Original Effective Date: August 12,
More informationSynopsis of Management on Ventricular arrhythmias. M. Soni MD Interventional Cardiologist
Synopsis of Management on Ventricular arrhythmias M. Soni MD Interventional Cardiologist No financial disclosure Premature Ventricular Contraction (PVC) Ventricular Bigeminy Ventricular Trigeminy Multifocal
More informationUnusual Tachycardia Association In A patient Without Structural Heart Disease
www.ipej.org 233 Case Report Unusual Tachycardia Association In A patient Without Structural Heart Disease Eduardo Arana-Rueda, Alonso Pedrote, Lorena Garcia-Riesco, Manuel Frutos-Lopez, Juan A. Sanchez-Brotons
More informationRecurrent Implantable Defibrillator Discharges (ICD) Discharges ICD Storm
Recurrent Implantable Defibrillator Discharges (ICD) Discharges ICD Storm Guy Amit, MD, MPH Soroka University Medical Center Ben-Gurion University of the Negev Beer-Sheva, Israel Disclosures Consultant:
More informationStage I: Binning Dashboard
Stage I: Binning Dashboard P[ GENE/GENE PANEL: KCNQ1, KCNH2, SCN5A DISORDER: Romano-Ward Long QT Syndrome HGNC ID: 6294, 6251, 10593 OMIM ID: 192500, 613688, 603830 ACTIONABILITY PENETRANCE 1. Is there
More informationIN THE NAME OF GOD. Dr.Sima Sayah
IN THE NAME OF GOD Dr.Sima Sayah Epidemiology: Prevalence: ranging from 0.14% in the japanese to 0.61% in europeans & may reach to 3% in southeast Asia. In up to 60% of patients,the disease can be sporadic.
More informationCongenital long QT syndrome of particularly malignant course connected with so far unknown mutation in the sodium channel SCN5A gene
CASE REPORT Cardiology Journal 2013, Vol. 20, No. 1, pp. 78 82 10.5603/CJ.2013.0012 Copyright 2013 Via Medica ISSN 1897 5593 Congenital long QT syndrome of particularly malignant course connected with
More informationJ-wave syndromes: update on ventricular fibrillation mechanisms
J-wave syndromes: update on ventricular fibrillation mechanisms Michael Nabauer University of Munich, Germany 28.8.2011 I have no conflicts of interest ECG labelling by Einthoven Circ 1998 Osborn wave
More information2017 AHA/ACC/HRS Ventricular Arrhythmias and Sudden Cardiac Death Guideline. Top Ten Messages. Eleftherios M Kallergis, MD, PhD, FESC
2017 AHA/ACC/HRS Ventricular Arrhythmias and Sudden Cardiac Death Guideline Top Ten Messages Eleftherios M Kallergis, MD, PhD, FESC Cadiology Department - Heraklion University Hospital No actual or potential
More informationSEMINAIRES IRIS. Sudden cardiac death in the adult. Gian Battista Chierchia. Heart Rhythm Management Center, UZ Brussel. 20% 25% Cancers !
Sudden cardiac death in the adult Gian Battista Chierchia. Heart Rhythm Management Center, UZ Brussel.! " # $ % Cancers National Vital Statistics Report, Vol 49 (11), Oct. 12, 2001. 20% 25% State-specific
More informationWoo-Sik Yu, M.D. 1, Tae-Hoon Kim, M.D. 2, Jee won Suh, M.D. 1, Seunghwan Song, M.D. 1, Chang Young Lee, M.D. 1, Boyoung Joung, M.D., Ph.D.
Korean J Thorac Cardiovasc Surg 2015;48:220-224 ISSN: 2233-601X (Print) ISSN: 2093-6516 (Online) Case Report http://dx.doi.org/10.5090/kjtcs.2015.48.3.220 Thoracoscopic Left Cardiac Sympathetic Denervation
More informationTachycardia Devices Indications and Basic Trouble Shooting
Tachycardia Devices Indications and Basic Trouble Shooting Peter A. Brady, MD., FRCP Cardiology Review Course London, March 6 th, 2014 2011 MFMER 3134946-1 Tachycardia Devices ICD Indications Primary and
More informationAsymptomatic Long QT. Prof. Dr. Martin Borggrefe Mannheim
Asymptomatic Long QT Prof. Dr. Martin Borggrefe Mannheim QT interval Distribution of QTc intervals in large population-based studies Viskin S, Heart Rhythm 2009; 6: 711-715 QT interval Distribution of
More informationPatient Resources: Cardiac Channelopathies
Patient Resources: Cardiac Channelopathies Overview of Cardiac Channelopathies: CPVT, Long QT Syndrome and Brugada Syndrome Heart muscle cells contract because of movement of certain molecules (called
More informationInvasive Risk Stratification: When is it needed?
Inherited Cardiomyopathies and Channelopathies: Who is at risk for Sudden Cardiac Death? Invasive Risk Stratification: When is it needed? Hung-Fat Tse, MD, PhD Department of Medicine The University of
More informationClinical Policy: Holter Monitors Reference Number: CP.MP.113
Clinical Policy: Reference Number: CP.MP.113 Effective Date: 05/18 Last Review Date: 04/18 Coding Implications Revision Log Description Ambulatory electrocardiogram (ECG) monitoring provides a view of
More informationFANS Long QT Syndrome Investigation Protocol (including suspected mutation carriers)
Clinical Features FANS Long QT Syndrome Investigation Protocol (including suspected mutation carriers) History Syncope or presyncope compatible with ventricular tachyarrhythmia, especially relating to
More informationAntiarrhythmic Drugs
Antiarrhythmic Drugs DR ATIF ALQUBBANY A S S I S T A N T P R O F E S S O R O F M E D I C I N E / C A R D I O L O G Y C O N S U L T A N T C A R D I O L O G Y & I N T E R V E N T I O N A L E P A C H D /
More informationThe ajmaline challenge in Brugada syndrome: Diagnostic impact, safety, and recommended protocol
European Heart Journal (2003) 24, 1104 1112 The ajmaline challenge in Brugada syndrome: Diagnostic impact, safety, and recommended protocol Sascha Rolf*, Hans-Jürgen Bruns, Thomas Wichter, Paulus Kirchhof,
More informationBrugada Syndrome: An Update
Brugada Syndrome: An Update Osama Diab Associate professor of Cardiology Ain Shams university, Cairo, Egypt Updates Mechanism and Genetics Risk stratification Treatment 1 Brugada syndrome causes 4 12%
More information3/17/2014. NCDR-14 ICD Registry WS # 24 Case Scenarios Including Syndromes w/ Risk of Sudden Death. Objectives
NCDR-14 ICD Registry WS # 24 Case Scenarios Including Syndromes w/ Risk of Sudden Death Denise Pond BSN, RN The following relationships exist related to this presentation: No Disclosures Objectives Discuss
More informationProtocol. Genetic Testing for Cardiac Ion Channelopathies
Protocol Genetic Testing for Cardiac Ion Channelopathies (20443) Medical Benefit Effective Date: 04/0/8 Next Review Date: /8 Preauthorization Yes Review Dates: 05/09, 05/0, 03/, 03/2, 03/3, 03/4, 03/5,
More informationCase Presentation. Asaad Khan University College Hospital Galway Rep of Ireland
Case Presentation Asaad Khan University College Hospital Galway Rep of Ireland Case History 32 male Married Working as a chartered accountant P/C:Admitted to a Regional hospital to be investigated for
More informationGenetic Testing for Cardiac Ion Channelopathies. Description
Genetic Testing for Cardiac Ion Page: 1 of 30 Last Review Status/Date: March 2017 Genetic Testing for Cardiac Ion Description Genetic testing is available for patients suspected of having cardiac ion channelopathies
More informationWhat is New in CPVT? Diagnosis Genetics Arrhythmia Mechanism Treatment. Andreas Pflaumer
What is New in CPVT? Diagnosis Genetics Arrhythmia Mechanism Treatment Andreas Pflaumer Diagnosis of CPVT Induction of different types of VES or VT by exercise or catecholamines AND exclusion of of other
More informationEKG Rhythm Interpretation Exam
as EKG Rhythm Interpretation Exam Name: Date: ID# Unit Assume each strip is a 6 second strip. Passing is 80%. 1. Identify the following rhythm: a. Asystole b. Ventricular fibrillation c. Atrial fibrillation
More informationDescription. Page: 1 of 31. Genetic Testing for Cardiac Ion Channelopathies. Last Review Status/Date: December 2015
Genetic Testing for Cardiac Ion Last Review Status/Date: December 2015 Genetic Testing for Cardiac Ion Description Page: 1 of 31 Genetic testing is available for patients suspected of having cardiac ion
More informationUNDERSTANDING YOUR ECG: A REVIEW
UNDERSTANDING YOUR ECG: A REVIEW Health professionals use the electrocardiograph (ECG) rhythm strip to systematically analyse the cardiac rhythm. Before the systematic process of ECG analysis is described
More informationQT Interval: The Proper Measurement Techniques.
In the name of God Shiraz E-Medical Journal Vol. 11, No. 2, April 2010 http://semj.sums.ac.ir/vol11/apr2010/88044.htm QT Interval: The Proper Measurement Techniques. Basamad Z*. * Assistant Professor,
More informationNATIONAL INSTITUTE FOR HEALTH AND CLINICAL EXCELLENCE
NATIONAL INSTITUTE FOR HEALTH AND CLINICAL EXCELLENCE Implantable cardioverter defibrillators for the treatment of arrhythmias and cardiac resynchronisation therapy for the treatment of heart failure (review
More informationChapter 9. Learning Objectives. Learning Objectives 9/11/2012. Cardiac Arrhythmias. Define electrical therapy
Chapter 9 Cardiac Arrhythmias Learning Objectives Define electrical therapy Explain why electrical therapy is preferred initial therapy over drug administration for cardiac arrest and some arrhythmias
More informationProfessor Eric Schulze-Bahr
No CoI. Professor Eric Schulze-Bahr Institute for Genetics of Heart Diseases Department of Cardiology and Angiology University Hospital Münster / Germany ICD therapy in asymptomatic or borderline LQTS
More informationShort QT Syndrome: Pharmacological Treatment
Journal of the American College of Cardiology Vol. 43, No. 8, 2004 2004 by the American College of Cardiology Foundation ISSN 0735-1097/04/$30.00 Published by Elsevier Inc. doi:10.1016/j.jacc.2004.02.034
More informationECG Workshop. Carolyn Shepherd And Anya Horne UWE Principles of Cardiac Care
ECG Workshop Carolyn Shepherd And Anya Horne UWE Principles of Cardiac Care ECG workshop case study1 44 Year old male. Reports SOB, Lethargy, tiredness. PMH: Hypertension, nil else. What tests? What treatment?
More informationIndex. cardiacep.theclinics.com. Note: Page numbers of article titles are in boldface type.
Note: Page numbers of article titles are in boldface type. A AEDs. See Automated external defibrillators (AEDs) AF. See Atrial fibrillation (AF) Age as factor in SD in marathon runners, 45 Antiarrhythmic
More informationPattarapong Makarawate MD, FHRS Assistant Professor. Division Of Cardiology Faculty of Medicine, Khon Kaen University
Pattarapong Makarawate MD, FHRS Assistant Professor. Division Of Cardiology Faculty of Medicine, Khon Kaen University 1. Important and impact of ICD in primary prevention 2. Risk stratification for ICD
More informationSUDDEN CARDIAC DEATH(SCD): Definition
SUDDEN CARDIAC DEATH EPIDEMIOLOGY, PATHOPHYSIOLOGY, PREVENTION & THERAPY Hasan Garan, M.D. Columbia University Medical Center SUDDEN CARDIAC DEATH(SCD): Definition DEATH DUE TO A CARDIAC CAUSE IN A CLINICALLY
More informationStep by step approach to EKG rhythm interpretation:
Sinus Rhythms Normal sinus arrhythmia Small, slow variation of the R-R interval i.e. variation of the normal sinus heart rate with respiration, etc. Sinus Tachycardia Defined as sinus rhythm with a rate
More informationAdult Complexities of the Channelopathies
Adult Complexities of the Channelopathies Andrew Krahn MD FHRS Sauder Family and Heart and Stroke Foundation Chair in Cardiology Paul Brunes Chair in Heart Rhythm Disorders University of British Columbia
More informationRipolarizzazione precoce. Torino, 24th October Non così innocente come si pensava
Asymptomatic inherited arrhythmia syndromes: Drug induced Brugada Syndrome: when a prophylactic ICD is indicated? how high (or low) is QT the risk? Asymptomatic short Ripolarizzazione precoce. Torino,
More informationSUDDEN CARDIAC DEATH(SCD): Definition
SUDDEN CARDIAC DEATH EPIDEMIOLOGY, PATHOPHYSIOLOGY, PREVENTION & THERAPY Hasan Garan, M.D. Columbia University Medical Center SUDDEN CARDIAC DEATH(SCD): Definition DEATH DUE TO A CARDIAC CAUSE IN A CLINICALLY
More information