Is this syncope worrisome?

in Resident Report
May 8th, 2012

A case of a 58yom with a history of HTN who presented after a syncopal episode at Logan airport after eating sushi with wasabi. He felt faint and diaphoretic and like his throat was closing and then had a brief LOC after which he felt fine again. He had no visible seizure activity. His exam was unremarkable with negative orthostatic vital signs. His EKG had a classic pattern of a RSR’ in V2 & V3 with ST segment elevation and T-wave inversion in V1-V3 (also possibly a terminal S-wave in the lateral leads). This pattern is classic Brugada pattern. The pattern, along with his unexplained syncope, diagnose him with Brugada Syndrome. Brugada pattern without syndrome is a benign condition; however, brugada syndrome has a relatively high incidence of sudden death due to VF/VT and these patients need ICD implantation as primary prevention. Remember that unexplained syncope, syncope in older adults or recent prior episodes of syncope are concerning for pathology. Remember also that orthostatics and an EKG have high yield and low cost in the diagnosis of the etiology of syncope.

There are 3 types of Brugada syndrome, however type I is most common. It is related to a loss of function mutation in the SCN5A gene that encodes the myocyte sodium channel, leading to a shorter refractory period and conduction blockage which can precipitate a local reentry and subsequent VF/VT. First degree relatives should be screened with EKGs. Brugada syndrome (differentiated from the EKG pattern alone, which can be induced by fever or other sympathetic surges) is diagnosed by the EKG findings AND one of the following:

a)  Documented ventricular fibrillation
b)  Self-terminating polymorphic ventricular tachycardia (VT)
c)  Family history of sudden cardiac death at <45 years
d)  Type 1 ST segment elevation in family members
e)  Electrophysiologic inducibility of VT
f)  Unexplained syncope suggestive of a tachyarrhythmia
g)  Nocturnal agonal respiration

Arrhythmia inducing medications in Brugada syndrome are listed here: http://www.brugadadrugs.org/

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