Management of Wolff-Parkinson-White Tachyarrhythmia Presenting as Syncope with Seizure- like Activity * * Samuel Kaplan, BS and Lindsey Spiegelman, MD *University of California, Irvine, Department of Emergency Medicine, Orange, CA Correspondence should be addressed to Samuel Kaplan at [email protected] Submitted: August 15, 2017; Accepted: September 13, 2017; Electronically Published: October 15, 2017; https://doi.org/10.21980/J8534P Copyright: © 2017 Kaplan, et al. This is an open access article distributed in accordance with the terms of the Creative Commons Attribution (CC BY 4.0) License. See: http://creativecommons.org/licenses/by/4.0/ Empty Line Calibri Size 12 Empty Line Calibri Size 12 ABSTRACT: Audience: Emergency medicine residents and medical students. Introduction: An estimated 3% of the United States population suffers from recurrent convulsive episodes that are most often attributed to primary epileptic seizures.1 However, recent studies have estimated about 20%-30% of such episodes are associated with occult cardiac etiology,2 which carry one-year mortality rates of up to 30%.3 Cardiogenic cerebral hypoxia has been associated with a wide variety of neurologic disturbances, including dizzy spells, headache, syncope, focal motor deficit, generalized tonic-clonic seizure, confusion, dementia, and psychosis.4 Convulsive activity has tentatively been ascribed to the ensuing activation of the medullary reticular formation.5,6 This scenario is based on a patient that presented to University of California Irvine Medical Center Emergency Department in April 2017 who, following witnessed seizure-like episodes, was diagnosed with underlying Wolff-Parkinson-White (WPW) disorder. WPW is a congenital condition involving aberrantly conductive cardiac tissue between the atria and the ventricles that provides a pathway for a reentrant tachycardia circuit and ventricular pre-excitation.7 Diagnosis is primarily based on the presence of a short PR interval and delta waves on electrocardiography.8 While definitive treatment is catheter-based radiofrequency ablation of the accessory pathway, the hallmark of acute management is vagal maneuvers and antiarrhythmic drugs in the symptomatic but hemodynamically stable patient, and synchronized cardioversion in the unstable patient.9 WPW is thought to affect between 0.1% and 0.3% of the population, and while the usual clinical course is benign, sudden cardiac death occurs in about 3%-4% of such patients.7,10 One survey found 19% of patients with WPW had a history of syncopal episodes;11 however, precise prevalence surveys of WPW-associated seizure-like episodes are lacking in the current literature. This case illustrates the possibility of cardiogenic etiology underlying any convulsive or syncopal event, and thus the importance of its prompt detection and treatment. 38 Objectives: At the end of this simulation session the learner will: 1) Recognize clinical history suggestive of cardiogenic syncope, 2) recognize clinical evidence of Wolff-Parkinson-White syndrome, 3) promptly and appropriately treat unstable WPW tachyarrhythmia. Method: Moderate-fidelity simulation. Topics: Seizure, arrhythmia, Wolff-Parkinson-White (WPW), simulation, cardiac electrophysiology, adult resuscitation. Section break 39 USER GUIDE List of Resources: is allows the instructor to review any cognitive, diagnostic or Abstract 38 management errors, problems with communication, or points User Guide 40 of confusion. Instructor Materials 21 Recommended pre-reading for instructor: Operator Materials 28 • Di Biase L, Walsh E. Epidemiology, clinical Debriefing and Evaluation Pearls 30 manifestations, and diagnosis of the Wolff-Parkinson- Simulation Assessment 33 White syndrome. In: Downey BC, ed. UpToDate. Waltham, MA: UpToDate Inc. https://www.uptodate.com/contents/wolff-parkinson- Learner Audience: white-syndrome-anatomy-epidemiology-clinical- Medical students, interns, junior residents, senior residents, mid-level providers manifestations-and-diagnosis. Updated November 2016. Accessed October 1, 2017. Time Required for Implementation: • Di Biase L, Walsh E. Treatment of symptomatic Instructor Preparation: 15-30 minutes arrhythmias associated with the Wolff-Parkinson- Time for case: 15-20 minutes White syndrome. In: Downey BC, ed. UpToDate. Time for debriefing: 15-30 minutes Waltham, MA: UpToDate Inc. https://www.uptodate.com/contents/treatment-of- Recommended Number of Learners per Instructor: symptomatic-arrhythmias-associated-with-the-wolff- 2-5 parkinson-white-syndrome. Updated September 2016. Accessed October 1, 2017. Topics: • Page RL, Joglar JA, Caldwell MA, Calkins H, Conti JB, Seizure, arrhythmia, Wolff-Parkinson-White (WPW), Deal BJ, et al. 2015 ACC/AHA/HRS guideline for the simulation, cardiac electrophysiology, adult resuscitation. management of adult patients with supraventricular tachycardia: a report of the American College of Objectives: Cardiology/American Heart Association Task Force on By the end of this simulation session, the learner will be able clinical practice guidelines and the Heart Rhythm to: Society. J Am Coll Cardiol. 2016;67(13):e27-e115. doi: 1. Recognize clinical history suggestive of cardiogenic 10.1016/j.jacc.2015.08.856 syncope • Neumar RW, Shuster M, Callaway CW, Gent LM, Atkins 2. Recognize clinical evidence of Wolff-Parkinson- DL, Bhanji F, et al. Part 1: executive summary: 2015 White (WPW) syndrome American Heart Association guidelines update for 3. Promptly and appropriately treat unstable WPW cardiopulmonary resuscitation and emergency tachyarrhythmia cardiovascular care. Circulation. 2015;132(18 Suppl 2):S315-S167. doi: 10.1161/CIR. 0000000000000252 Linked objectives and methods: WPW syndrome is an uncommon but dangerous etiology of Results and tips for successful implementation: syncope and must be treated promptly, especially when the • This can be completed on a high- or moderate-fidelity patient is hemodynamically unstable. Through this simulation, simulation or could be incorporated as a mock oral learners will practice evaluating and managing an acutely ill board case. The patient, accompanied by his girlfriend, patient with history suggestive of seizure-like episode but with should initially present with altered mental status an underlying cardiac arrhythmia, namely WPW. If appropriate following seizure-like activity, which leaves a wide diagnosis and treatment are delayed learners will experience a differential. This simulation case was partially based on decompensating patient at risk of cardiac arrest and death. an actual patient case. • Instructors can provide more information suggestive of More specifically, by running through the simulation learners cardiac syncope or less depending on the level of the should recognize historical and physical exam findings learner. suggestive of cardiogenic syncope (objective 1), and recognize • This case was piloted with 14 emergency medicine Wolff-Parkinson-White syndrome on electrocardiogram (ECG) residents and medical students. The more advanced (objective 2). When they patient goes into an unstable learners kept their differential broad and considered a tachyarrhythmia they must appropriately treat it or the the cardiogenic cause while many more junior learners patient will decompensate (objective 3). The debriefing session anchored on the seizure and did not diagnose eturn: Calibri Size 10 40 USER GUIDE cardiogenic syncope until the tachyarrhythmia syndrome: results of a prospective long-term occurred. Overall, the case was well received and electrophysiological follow-up study. Circulation. learners noted they appreciated the reminder that 2012;125(5):661-668. doi: cardiogenic syncope must be on the differential for 10.1161/CIRCULATIONAHA.111.065722 patients with a chief complaint of seizure. 11. Goudevenos JA, Katsouras CS, Graekas G. Ventricular pre- excitation in the general population: a study on the mode References/suggestions for further reading: of presentation and clinical course. Heart. 2000;83(1):29- 1. Hauser WA, Kurland LT. The epidemiology of epilepsy in 34. doi: 10.1136/heart.83.1.29 Rochester, Minnesota, 1935 through 1967. Epilepsia. Section Break 1975;16(1):1-66. 2. Kanjwal K, Karabin B, Kanjwal Y, Grubb BP. Differentiation of convulsive syncope from epilepsy with an implantable loop recorder. Int J Med Sci. 2009;6(6):296-300. 3. Kapoor WN, Karpf M, Wieand S, Peterson JR, Levey GS. A prospective evaluation and follow-up of patients with syncope. N Engl J Med. 1983;309(4):197-204. doi: 10.1056/NEJM198307283090401 4. Schott GD, McLeod AA, Jewitt DE. Cardiac arrhythmias that masquerade as epilepsy. Br Med J. 1977;1(6074):1454- 1457. 5. Lempert T, Bauer M, Schmidt D. Syncope: a videometric analysis of 56 episodes of transient cerebral hypoxia. Ann Neurol. 1994:36(2):233-237. doi: 10.1002/ana.410360217 6. Dell P, Hugelin A, Bonvallet M. Effects of hypoxia on the reticular and cortical diffuse systems. In: Gastaut H, Meyer JS, eds. Cerebral Anoxia and the Electroencephalogram. Springfield, IL: Charles C Thomas; 1961:46-58. 7. Cohen MI, Triedman JK, Cannon BC, Davis AM, Drago F, Janoursek J, et al. PACES/HRS expert consensus statement on the management of the asymptomatic young patient with a Wolff-Parkinson-White (WPW, ventricular preexcitation) electrocardiographic pattern: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology Foundation (ACCF), the