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A Young Man With Syncope

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his early 20s presented with suspected syncope and shaking with no significant medical history. The patient fell asleep and his girlfriend tried to wake him, but he started shaking and slumped over on the ground. He denied confusion afterward but did not feel well with nausea, flushing, chest tightness, and a rapid heart rate. About 10 minutes later, he had a second syncopal episode for 2 to 3 minutes with more than 20 muscle jerks. On presentation at the emergency department, he was afebrile, blood pressure was 120/59 mm Hg, heart rate was 85 beats per min, respiratory rate was 16 breaths per min, and oxygen saturation was 96% on room air. His cardiac examination revealed normal heart sounds with no murmurs.

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Brugada type 1 syndrome with unexplained syncope

C. Implantable cardioverter defibrillator

The key to the correct diagnosis in this case was the ECG that was consistent with spontaneous Brugada type 1 morphology with more than 2-mm coved ST-segment elevation in leads V1 and V2 (Figure). Recognition of the Brugada type 1 pattern as a potential cause of cardio-arrhythmogenic syncope is important.

The incidence of the Brugada type 1 ECG pattern in the US is thought to be as low as 0.03%.1 The proposed Shanghai Score System2 can be used for the diagnosis of Brugada syndrome, which relies on ECG, clinical history, family history, or genetic test results. A score of more than 3.5 points is thought to be probable and/or definite Brugada. Brugada type 1 can present asymptomatically, in which case the management is observation. However, in those with symptomatic features, such as unexplained cardiac arrest or polymorphic ventricular tachycardia or ventricular fibrillation arrest, nocturnal agonal respirations, atrial fibrillation/flutter in patients younger than 30 years of age, or syncope of uncertain etiology, further evaluation is needed.

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Article Information

Corresponding Author: Asad J. Torabi, MD, Division of Cardiology, Krannert Institute of Cardiology at Indiana University School of Medicine, 1800 N Capital Ave, Indianapolis, IN 46202 (ajtorabi@iupui.edu).

Published Online: June 8, 2022. doi:10.1001/jamacardio.2022.1313

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank Jeffrey Mossler, MD, Indiana University School of Medicine, Indianapolis, for his assistance in reviewing the manuscript; he did not receive financial compensation for his contribution. We also the patient for granting permission to publish this information.

References
1.
Mizusawa  Y , Wilde  AA .  Brugada syndrome.   Circ Arrhythm Electrophysiol. 2012;5(3):606-616. doi:10.1161/CIRCEP.111.964577PubMedGoogle ScholarCrossref
2.
Kawada  S , Morita  H , Antzelevitch  C ,  et al.  Shanghai score system for diagnosis of Brugada syndrome: validation of the score system and system and reclassification of the patients.   JACC Clin Electrophysiol. 2018;4(6):724-730. doi:10.1016/j.jacep.2018.02.009PubMedGoogle ScholarCrossref
3.
Kusumoto  FM , Bailey  KR , Chaouki  AS ,  et al.  Systematic review for the 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.   Heart Rhythm. 2018;15(10):e253-e274. doi:10.1016/j.hrthm.2017.10.037PubMedGoogle ScholarCrossref
4.
Peterson  PN , Greenlee  RT , Go  AS ,  et al.  Comparison of inappropriate shocks and other health outcomes between single—and dual-chamber implantable cardioverter-defibrillators for primary prevention of sudden cardiac death: results from the cardiovascular research network longitudinal study of implantable cardioverter-defibrillators.   J Am Heart Assoc. 2017;6(11):e006937. doi:10.1161/JAHA.117.006937PubMedGoogle ScholarCrossref
5.
Knops  RE , Olde Nordkamp  LRA , Delnoy  PHM ,  et al; PRAETORIAN Investigators.  Subcutaneous or transvenous defibrillator therapy.   N Engl J Med. 2020;383(6):526-536. doi:10.1056/NEJMoa1915932PubMedGoogle ScholarCrossref
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