Catecholaminergic Polymorphic Ventricular Tachycardia

CME REVIEW ARTICLE

Jessica J. Wall, MD, MPH* and Ramesh V.Iyer, MD†

department. What is the likely diagnosis, and what is your manage- Abstract: Catecholaminergic polymorphic ventricular tachycardia is a ment in the emergency department? rare cause of exercise-induced arrhythmia and sudden cardiac death in the pediatric patient. This arrhythmia is difficult to diagnose in the emer- DEFINITION AND EPIDEMIOLOGY gency department, given the range of presentations; thus, a familiarity with and high index of suspicion for this pathology are crucial. Furthermore, Catecholaminergic polymorphic ventricular tachycardia (CPVT) recognition of the characteristic electrocardiogram findings and knowledge is a cardiac arrhythmia characterized by an emotion- or exercise- 1 of the management of the symptomatic patient are necessary, given the risk induced polymorphic ventricular tachycardia. Although previ- 2 of arrhythmia recurrence and cardiac arrest. In this review, we discuss the ously described in case reports in 1975, CPVT was defined by 1 presentation, differential diagnosis, and management of catecholaminergic Leenhardt et al in 1995 as an etiology of polymorphic ventricular polymorphic ventricular tachycardia for the emergency care provider. tachycardia with exercise in patients with an otherwise normal resting ECG and structurally normal heart. The characteristic ECG Key Words: catecholaminergic polymorphic ventricular tachycardia, finding when symptomatic is bidirectional ventricular tachycar- sudden cardiac arrest dia, as seen in Figure 2. While the exact incidence of CPVT is un- (Pediatr Emer Care 2017;33: 427–433) known, it is estimated to be 1 in 10,000.3,4 When left untreated, symptomatic CPVT has a mortality of 30% by the age of 40 years.5,6 Typical age at presentation ranges from early child- TARGET AUDIENCE hood to early adulthood, with an average between 11 and 21 years; This CME activity is intended for pediatric emergency medi- however, incident symptomatic cases have been reported in adults 1,5,7 cine physicians, emergency medicine physicians, and pediatricians. up to 40 years old. Furthermore, CPVT is often misdiagnosed initially as vasovagal syncope, seizure disorder, and other arrhythmic conditions such as long QT syndrome (LQTS), resulting in an LEARNING OBJECTIVES average delay to diagnosis of up to 2 years.7 After completion of this article, the reader should be able to: PATHOPHYSIOLOGY AND GENETICS 1. Define catecholaminergic polymorphic ventricular tachycardia While the overarching mechanism of CPVT is related to an 2. Review pathophysiology of catecholaminergic polymorphic increase in the release of calcium (Ca2+) from the junctional sarco- ventricular tachycardia; plasmic reticulum (SR) into the cytoplasm of the cardiac myocyte, 3. Review presentation and diagnosis of catecholaminergic poly- there are multiple mechanisms and concomitant gene mutations morphic ventricular tachycardia; associated with this pathology.6 Understanding the regulation of 4. Review electrocardiogram findings indicative of catecholamin- Ca2+ release in a myocyte is crucial to understanding this patho- ergic polymorphic ventricular tachycardia; and physiology (Fig. 3). In response to the action potential propagat- 5. Discuss current consensus guidelines for the acute and ing throughout the myocardium, calcium enters the cell through chronic management strategies catecholaminergic polymorphic the voltage-gated Ca2+ channel (L-type channels). Once in the cell, ventricular tachycardia. the elevated Ca2+ level induces the ryanodine (RyR2) channel lo- cated in the junctional SR to release calcium from the SR into the cytoplasm of the cell, so-called “Ca2+-induced Ca2+ release.” The 13-year old child presents to the emergency department after resultant elevation in intracellular Ca2+ induces contraction of the A collapsing while running in a soccer game. He required car- sarcomere by binding to troponin in the myofilaments. The RyR2 diopulmonary resuscitation and 1 defibrillation by an automated channel is additionally activated by stimulation from β-adrenergic external defibrillator prior to emergency medical services arrival. receptors via a cAMP mechanism, increasing Ca2+ in the SR. The His parents report that he has collapsed previously, but he always Ca2+ is then removed from the cytoplasm by Ca2+ ATPase and sodium-dependent mechanisms transporting it back into the SR recovered within a few minutes. The paramedics show you the – electrocardiogram (ECG) (Fig. 1) obtained en route to the emergency and the extracellular space.8 10 There are 4 genes that have been directly linked to CPVT, all of which affect Ca2+ release in the cardiac myocyte. Currently, † only 60% of cases of CPVT are found to have a genetic mutation, *Fellow (Wall), Division of Emergency Medicine and Associate Clinical 11 Professor (Iyer), Division of Cardiology, Children’s Hospital of Philadelphia, and additional genes are currently under investigation. The first Philadelphia, PA. identified genetic mutation was described in the cardiac ryanodine The authors, faculty, and staff in a position to control the content of this CME receptor (RYR2 or CPVT1) gene, which accounts for 50% to 55% activity and their spouses/life partners (if any) have disclosed that they have of CPVT cases.1,3,12 This mutation is autosomal dominant and re- no financial relationships with, or financial interest in, any commercial 2+ organizations pertaining to this educational activity. sults in diastolic “leak” of Ca into the SR leading to elevated Reprints: Jessica J. Wall, MD, MPH, Division of Emergency Medicine, Ca2+ levels in the cytoplasm. This effect is noted to be more pro- Children’s Hospital of Philadelphia, 3501 Civic Center Blvd, 9th Floor/ nounced in high β-adrenergic states and hence the association of CTRB 9013 B, Philadelphia, PA 19104 2,12,13 (e‐mail: [email protected]). arrhythmias with exercise. The second genetic mutation Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved. identified in CPVT patients is the CASQ2 (CPVT2) gene, which ISSN: 0749-5161 encodes cardiac calsequestrin, a Ca2+-buffering protein in the SR

Pediatric Emergency Care • Volume 33, Number 6, June 2017 www.pec-online.com 427 Wa ll an d I ye r Pediatric Emergency Care • Volume 33, Number 6, June 2017

FIGURE 1. Electrocardiogram of a 13-year-old boy with CPVT. that inhibits the RYR2 protein.1,14 This mutation is autosomal re- ventricular tachycardia can be diagnosed further via genetic test- cessive and accounts for only 2% to 5% of CPVT patients.3,4 The ing in the absence of symptoms at the time of presentation.4,18 third gene identified in association with CPVT is CALM1, which A family history of sudden unexpected death in a relative younger is autosomal dominant and encodes calmodulin, a protein that binds than 40 years, an ECG showing exercise-induced ventricular ectopy, calcium and stabilizes the RyR2 channel and accounts for less or a seizure during exercise (common misdiagnosis in CPVT) should than 1% of cases.3,5,6,15 Finally, in 2012, the gene TRDN was also raise suspicion of CPVT. identified in 2 families with CPVT.1,5,7,16 TRDN encodes triadin, While genetic testing has become a standard in diagnosis for a protein that links RyR2 and celsequestrin in the SR. CPVT, Holter monitoring, exercise stress testing (EST), and iso- proterenol or epinephrine challenge may be useful in making the diagnosis and differentiating from LQTS. The baseline ECG in PRESENTATION AND DIAGNOSIS a CPVT patient typically is normal, occasionally marked by mild The most common clinical presentation of CPVT is syncope, bradycardia.19 Progressive ventricular ectopy during EST may be followed by cardiac arrest as in our case presentation. However, it a hallmark for diagnosing these patients. Appearance of premature should be noted that given the ability to test for genetic mutations ventricular beats (PVCs), bigeminy or trigeminy, polymorphic associated with this pathology, asymptomatic patients (especially PVCs, and finally nonsustained to sustained bidirectional ventric- relatives of affected patients) are now being identified in up to ular tachycardia have been described in CPVT.1,3,20 The character- 19% of cases.1,5,7,17 In the largest cohort study to date, 64% of pa- istic bidirectional ventricular tachycardia is rarely found on ECG; tients presented with syncope, 33% with cardiac arrest, 7% with more common are polymorphic PVCs; thus, careful attention to palpitations, and 4% with chest pain.7–10 The average age at pre- the ECG and telemetry is necessary for clinical diagnosis. sentation is approximately 11 years old.7,11 Current guidelines recommend genetic testing for CPVT Clinical diagnosis is based on the documentation of a poly- genes in patients who exhibit symptoms or if there is a suspicious morphic ventricular tachycardia that is induced by adrenergic family history. In addition, once a pathogenic mutation is identi- stimuli (emotion or exercise) without any additional structural or fied, given the high risk to family members, complete cardiac electrical cardiac abnormality.4 Catecholaminergic polymorphic evaluation and testing including mutation-specific genetic testing are recommended in relatives of the index case.11 For the emergency provider, the basis for diagnosis of CPVT is heightened clinical awareness of the pathology and attention to the history of presentation. In general, patients who present at younger than 18 years with exertional syncope have a likelihood of a cardiac etiology of approximately 50%.21 Table 1 summarizes findings concerning for cardiac etiology and CPVT of patients presenting to the emergency department.7

ACUTE MANAGEMENT STRATEGIES At the outset, it is important to distinguish benign syncope (such as vasovagal syncope) from the arrhythmogenic syncope induced by exercise in conditions such as CPVT and LQTS. This can be done by taking a detailed history of the event with particular inquiry of prodromal symptoms (such as dizziness, lightheadedness, or visual or auditory stimuli) that may suggest a more benign etiology. Emergency management of symptomatic CPVTwith ongo- ing arrhythmia is guided by the presentation and stability of the patient. Polymorphic ventricular tachycardia of any etiology should be differentiated into pulseless patients and patients with a pulse, FIGURE 2. Example of bidirectional ventricular tachycardia in a which can further be subdivided into stable and unstable based on patient with CPVT. signs of hemodynamic compromise.

FIGURE 3. Components of calcium release unit (CRU) and mitochondria involved in excitation-contraction coupling. Interface between T-tubule and SR with RyR2 receptors (RyR2s) is circled in red. RyR2 indicates ryanodine receptor; Trdn, triadin; Calm1, calmodulin; Casq2, calsequestrin; SR, junctional SR; L-type Ca2+ channel, voltage-gated calcium channel; NCX, sodium-calcium exchanger. Calcium is released from the SR via RyR2 interaction with the myofilaments to cause muscle contraction (red arrows). During muscle relaxation, calcium reuptake into the SR and mitochondria occurs (green arrows). Reprinted (with modification) by permission from Macmillan Publishers Ltd (Bers DM. Cardiac excitation-contraction coupling. Nature 2002;415:198–205; copyright 2016).

Pulseless including synchronized cardioversion/defibrillation (polymorphic ventricular tachycardia may not permit identification of the QRS Pulseless patients, as in our introductory case, should be treated 23,25 following Pediatric Advanced Life Support and Advanced Cardiac for cardioversion; thus, defibrillation may be necessary), mag- – – 25 β Life Support guidelines.22 25 Specific therapies for polymorphic nesium administration (20 50 mg/kg), intravenous -blocker ventricular tachycardia include defibrillation (2–4upto10J/ therapy (propranolol 0.1 mg/kg up to 3 mg over 10 minutes or μ – μ kg),25 magnesium administration (20–50 mg/kg),25 intravenous esmolol 500- g/kg bolus followed by a drip of 50 300 g/kg 23 – 25 β-blocker therapy (esmolol 500 μg/kg bolus followed by a drip per minute), and amiodarone (5 mg/kg over 20 60 minutes). of 50–300 μg/kg per minute),23 andamiodarone(5mg/kg,may be repeated twice up to 15 mg/kg, maximum dose 300 mg).23,25 Exercise-Related Syncope Approximately half of pediatric patients who present with With a Pulse exercise-related syncope are found to have a cardiac etiology.21 The differential diagnoses for syncope with exertion include LQTS, Polymorphic ventricular tachycardia likely associated with Wolff-Parkinson-White syndrome, hypertrophic cardiomyopathy, CPVTwith a pulse should be managed with removal of catechol- pulmonary hypertension, Brugada, intermittent heart block, amine stimulus (such as pain or anxiety), β-blocker therapy, and 26 arrhythmogenic right ventricular dysplasia, CPVT, and rarely sedation to decrease catecholamine release when stable. The ba- neurocardiogenic syncope when all other etiologies have been sis of this management strategy is to neutralize the adrenergic ruled out.27,28 Tretter and Kavey29 proposed screening for exercise- stimulation from the RyR2 channel resulting in the abnormal 2+ related syncope with family history of cardiac events or sudden Ca release. Thus, it is imperative to reduce pain, treat anxiety/ unexplained death, abnormal physical examination, and abnormal agitation, and provide sedation for painful procedures to reduce ECG. If 1 or more of these parameters is positive in a child with the adrenergic surge that will potentiate the arrhythmia. syncope, screening for underlying cardiac disease should be un- In patients who are unstable and nearing pulseless arrest, dertaken. The etiology of exertional syncope may not be elucidated management is targeted at immediate cessation of the rhythm, in the emergency department, given limited testing; however, these patients should be further investigated with an exercise stress test, TABLE 1. Presenting Symptoms of CPVT echocardiography, and cardiology referral.

Syncope associated with emotional stress or physical exercise CHRONIC MANAGEMENT STRATEGIES Seizures Long-term management of CPVT is targeted at reduction in Palpitations adrenergic stimulation to the RyR2 channel with β-blockers and Chest pain with exercise lifestyle modifications. In patients who continue to be at risk of Cardiac arrest ventricular arrhythmia, an implantable defibrillator can be placed Family history of sudden unexplained death to prevent sudden cardiac arrest. ECG with ventricular ectopy Lifestyle modification is the first tenant of management in CPVT patients with the modification or cessation of exercise.7,18,30

Initial pharmacologic intervention includes β-blocker therapy strategies and outcomes from an international multicenter registry. Circ with propranolol or nadolol; however, there is a 25% treatment Arrhythm Electrophysiol. 2015;8:633–642. 4,7,18 failure with this intervention. In the setting of incomplete 8. Watanabe H, Knollmann BC. Mechanism underlying catecholaminergic control of the ventricular arrhythmia or intolerance of β-blocker polymorphic ventricular tachycardia and approaches to therapy. therapy, flecainide, which is a direct RyR2-channel blocker, may J Electrocardiol. 2011;44:1–6. be added. In the past, the calcium-channel blocker verapamil was 9. Fabiato A. Calcium-induced release of calcium from the cardiac included in the pharmacologic management of CPVT, but effec- sarcoplasmic reticulum. Am J Physiol. 1983;245:C1–C14. tiveness is controversial.4,18 10. Wehrens XH, Lehnart SE, Marks AR. Intracellular calcium release and Implantable cardiac defibrillator (ICD) placement is a class I cardiac disease. Annu Rev Physiol. 2005;67:69–98. recommendation for CPVT patients with a history of cardiac arrest, recurrent syncope, or polymorphic ventricular tachycardia de- 11. Ackerman MJ, Priori SG, Willems S, et al. HRS/EHRA expert consensus spite pharmacologic therapy.18,31 Even after placement of an ICD, statement on the state of genetic testing for the channelopathies and pharmacologic management needs to be optimized to reduce the cardiomyopathies this document was developed as a partnership between number of shocks a patient experiences. The last line of therapy for the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm. 2011;8:1308–1339. CVPT is left cardiac sympathetic denervation, which has been shown to be effective in patients who continue to experience syncope, 12. Priori SG, Napolitano C, Tiso N, et al. Mutations in the cardiac ryanodine polymorphic ventricular tachycardia, and/or several appropriate receptor gene (hRyR2) underlie catecholaminergic polymorphic ventricular – shocks from an ICD despite maximal pharmacologic therapy.18,32 tachycardia. Circulation. 2001;103:196 200. Current research involving gene therapy in a mouse CASQ2 13. Lehnart SE. Sudden death in familial polymorphic ventricular tachycardia CPVT model has shown promise putting a new treatment on the associated with calcium release channel (ryanodine receptor) leak. horizon with implications such as not only CPVT but also heart Circulation. 2004;109:3208–3214. 33,34 failure–associated arrhythmias. 14. Lahat H, Pras E, Olender T, et al. A missense mutation in a highly conserved region of CASQ2 is associated with autosomal recessive CONCLUSIONS catecholamine-induced polymorphic ventricular tachycardia in Bedouin families from Israel. Am J Hum Genet. 2001;69:1378–1384. For our introductory case, he converted to a normal sinus rhythm with intravenous β-blockade and anxiolysis. He was 15. Nyegaard M, Overgaard MT, Søndergaard MT, et al. Mutations in calmodulin cause ventricular tachycardia and sudden cardiac death. started on chronic β-blocker therapy with lifestyle modifications Am J Hum Genet. 2012;91:703–712. to limit intense physical activity, but was able to return to recrea- tional sports with an automated external defibrillator available at 16. Roux-Buisson N, Cacheux M, Fourest-Lieuvin A, et al. Absence of triadin, all times. Genetic testing was done for prognostication and to a protein of the calcium release complex, is responsible for cardiac arrhythmia with sudden death in human. Hum Mol Genet.2012;21: aidinthescreeningofhisimmediatefamilymembers. – Catecholaminergic polymorphic ventricular tachycardia is a 2759 2767. rare but life-threatening genetic condition characterized by ven- 17. Sy RW, Gollob MH, Klein GJ, et al. Arrhythmia characterization and tricular arrhythmia that can progress to polymorphic ventricular long-term outcomes in catecholaminergic polymorphic ventricular tachycardia in high adrenergic states. The emergency provider tachycardia. Heart Rhythm.2011;8:864–871. should have a high index of suspicion for this pathology in pa- 18. Priori SG, Blomström-Lundqvist C, Mazzanti A, et al. 2015 ESC tients presenting with exercise-induced syncope or seizure-like Guidelines for the management of patients with ventricular arrhythmias and activity in the presence of a normal examination, echocardiogram, the prevention of sudden cardiac death: the Task Force for the Management andbaselineECG. of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: REFERENCES Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J. 2015;36:2793–2867. 1. Leenhardt A, Lucet V,Denjoy I, et al. 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CME EXAMINATION JUNE 2017

Please mark your answers on the ANSWER SHEET.

Catecholaminergic Polymorphic Ventricular Tachycardia, Wall and Iyer.

1. What is the initial therapy for symptomatic catecholaminergic c. normal sinus rhythm polymorphic ventricular tachycardia (CPVT) with a pulse in d. first-degree atrioventricular block the emergency department? a. treatment of pain and/or anxiety 4. What presentation is concerning for CPVT? b. synchronized cardioversion a. first-time seizure with minimal tonic-clonic activity c. intravenous amiodarone b. syncope d. external pacing c. palpitations with exercise d. family history of CPVT 2. Which enzyme is not associated with pathway leading to CPVT? e. all of the above a. triadin b. calmodulin 5. What is the key to diagnosis of CPVT? c. troponin a. high index of suspicion and thorough history d. calsequestrin b. abnormal ECG c. echocardiogram 3. When asymptomatic, what electrocardiogram (ECG) finding is d. cardiac magnetic resonance imaging most characteristic of CPVT? a. ST elevation in the precordial leads b. prolongation of the QT segment

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