Brugada syndrome differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

Brugada syndrome should be differentiated from other cardiac disorders, electrolyte disturbances, and drug intoxication syndromes. The condition which most similarly presents to Brugada syndrome is arrhythmogenic right ventricular dysplasia, as they both cause sudden cardiac death in children. Brugada syndrome can be differentiated from arrhythmogenic right ventricular dysplasia by the genetic counterpart of SCN5A, the lack of structural abnormalities within the heart, the association with polymorphic ventricular tachycardia during sleep, and EKG changes that are enhanced by vagotonic agents.

Differentiating Brugada syndrome from other Diseases

Abnormalities that can lead to ST-segment elevation in the right precordial leads include the following:[1]

Differentiating Brugada Syndrome from Arrhythmogenic Right Ventricular Dysplasia

Although both Brugada syndrome and arrhythmogenic right ventricular dysplasia are associated with sudden cardiac death in young patients, the two syndromes are fairly easy to distinguish electrocardiographically and clinically.

Genetics

There is only one gene associated with Brugada syndrome, namely the SCN5A gene, and there is no overlap of the genetic abnormalities associated with arrhythmogenic right ventricular dysplasia.

Structural Abnormalities of the Right Ventricle

While Brugada syndrome is not associated with structural abnormalities in the right ventricle, arrhythmogenic right ventricular dysplasia is associated with fibrofatty infiltration.

Precipitant of Ventricular Arrhythmias

Arrhythmogenic right ventricular dysplasia is associated with monomorphic ventricular tachycardia with a left bundle branch morphology and is precipitated by catecholamines or exercise. In contrast, Brugada syndrome is associated with polymorphic ventricular tachycardia and occurs predominantly during sleep or rest.

Response to Pharmacologic Agents

The EKG abnormalities of Brugada syndrome are enhanced by vagotonic agents, beta-adrenergic blockers, and sodium channel blockers whereas the EKG changes of arrhythmogenic right ventricular dysplasia are constant and do not very with vagotonic agents, beta-adrenergic blockers, or sodium channel blockers.

References

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  2. Corrado D, Nava A, Buja G, Martini B, Fasoli G, Oselladore L, Turrini P, Thiene G. Familial cardiomyopathy underlies syndrome of right bundle branch block, ST segment elevation and sudden death. J Am Coll Cardiol. 1996; 27: 443–448.
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  11. Myers GB. Other QRS-T patterns that may be mistaken for myocardial infarction; IV. Alterations in blood potassium; myocardial ischemia; subepicardial myocarditis; distortion associated with arrhythmias. Circulation. 1950; 2: 75–93.
  12. Merrill JP, Levine HD, Somerville W, Smith S. Clinical recognition and treatment of acute potassium intoxication. Ann Intern Med. 1950; 33: 797–830.
  13. Ortega-Carnicer J, Benezet J, Ruiz-Lorenzo F, Alcázar R (2002). "Transient Brugada-type electrocardiographic abnormalities in renal failure reversed by dialysis". Resuscitation. 55 (2): 215–9. PMID 12413761. Retrieved 2012-10-13. Unknown parameter |month= ignored (help)
  14. Osborn JJ. Experimental hypothermia; respiratory and blood pH changes in relation to cardiac function. Am J Physiol. 1953; 175: 389–398.
  15. Noda T, Shimizu W, Tanaka K, Chayama K (2003). "Prominent J wave and ST segment elevation: serial electrocardiographic changes in accidental hypothermia". Journal of Cardiovascular Electrophysiology. 14 (2): 223. PMID 12693512. Retrieved 2012-10-13. Unknown parameter |month= ignored (help)
  16. Kataoka H. Electrocardiographic patterns of the Brugada syndrome in right ventricular infarction/ischemia. Am J Cardiol. 2000; 86: 1056.
  17. Wang K, Asinger RW, Marriott HJ (2003). "ST-segment elevation in conditions other than acute myocardial infarction". The New England Journal of Medicine. 349 (22): 2128–35. doi:10.1056/NEJMra022580. PMID 14645641. Retrieved 2012-10-13. Unknown parameter |month= ignored (help)
  18. Tarín N, Farré J, Rubio JM, Tuñón J, Castro-Dorticós J (1999). "Brugada-like electrocardiographic pattern in a patient with a mediastinal tumor". Pacing and Clinical Electrophysiology : PACE. 22 (8): 1264–6. PMID 10461308. Unknown parameter |month= ignored (help); |access-date= requires |url= (help)
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