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]
- Acute myocardial ischemia or infarction
- Acute myocarditis
- Acute pericarditis
- Acute pulmonary thromboemboli
- Arrhythmogenic right ventricular dysplasia / cardiomyopathy (ARVD/C)[2][3]
- Cardioversion. Brugada-like ECG changes can be observed briefly after direct-current cardioversion. It is currently unclear if this is a sign that the patient is a gene carrier for Brugada syndrome.[4][5][6]
- Cocaine intoxication
- Coronary spasm
- Dissecting aortic aneurysm[7]
- Duchenne muscular dystrophy[8]
- Early repolarization
- Friedreich ataxia
- Heterocyclic antidepressant overdose
- Hypercalcemia[9][10]
- Hyperkalemia[11][12][13]
- Hypothermia, can cause an Osborn wave on the ECG which can sometimes resemble Brugada syndrome[14][15]
- Left ventricular hypertrophy
- Pectus excavatum[16]
- Prinzmetal's angina[17]
- Mediastinal tumor compressing the right ventricular outflow tract (RVOT)
- Right or left bundle-branch block (atypical)
- Right ventricular infarction
- Right ventricular ischemia
- Right ventricular outflow tract compression due to a mediastinal tumoror hemopericardium[18][19]
- Thiamine deficiency[20]
- Various central and autonomic nervous system abnormalities
- [Other conditions that can lead to ST-segment elevation in the right precordial leads]]
- Early repolarization syndrome
- Other normal variants (particularly in males)
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.[21]
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
- ↑ Takehara N, Makita N, Kawabe J, Sato N, Kawamura Y, Kitabatake A, Kikuchi K (2004). "A cardiac sodium channel mutation identified in Brugada syndrome associated with atrial standstill". Journal of Internal Medicine. 255 (1): 137–42. PMID 14687250. Retrieved 2012-10-13. Unknown parameter
|month=ignored (help) - ↑ 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.
- ↑ Corrado D, Basso C, Buja G, Nava A, Rossi L, Thiene G. Right bundle branch block, right precordial ST-segment elevation, and sudden death in young people. Circulation. 2001; 103: 710–717.
- ↑ Kok LC, Mitchell MA, Haines DE, Mounsey JP, DiMarco JP (2000). "Transient ST elevation after transthoracic cardioversion in patients with hemodynamically unstable ventricular tachyarrhythmia". The American Journal of Cardiology. 85 (7): 878–81, A9. PMID 10758932. Retrieved 2012-10-14. Unknown parameter
|month=ignored (help) - ↑ Gurevitz O, Glikson M (2003). "Cardiac resynchronization therapy: a new frontier in the management of heart failure". The Israel Medical Association Journal : IMAJ. 5 (8): 571–5. PMID 12929296. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ Gurevitz O, Lipchenca I, Yaacoby E, Segal E, Perel A, Eldar M, Glikson M (2002). "ST-segment deviation following implantable cardioverter defibrillator shocks: incidence, timing, and clinical significance". Pacing and Clinical Electrophysiology : PACE. 25 (10): 1429–32. PMID 12418739. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ 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.
- ↑ Perloff JK, Henze E, Schelbert HR. Alterations in regional myocardial metabolism, perfusion, and wall motion in Duchenne muscular dystrophy studied by radionuclide imaging. Circulation. 1984; 69: 33–42.
- ↑ Douglas PS, Carmichael KA, Palevsky PM (1984). "Extreme hypercalcemia and electrocardiographic changes". The American Journal of Cardiology. 54 (6): 674–5. PMID 6475795. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ Sridharan MR, Horan LG (1984). "Electrocardiographic J wave of hypercalcemia". The American Journal of Cardiology. 54 (6): 672–3. PMID 6475794. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ 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.
- ↑ Merrill JP, Levine HD, Somerville W, Smith S. Clinical recognition and treatment of acute potassium intoxication. Ann Intern Med. 1950; 33: 797–830.
- ↑ 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) - ↑ Osborn JJ. Experimental hypothermia; respiratory and blood pH changes in relation to cardiac function. Am J Physiol. 1953; 175: 389–398.
- ↑ 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) - ↑ Kataoka H. Electrocardiographic patterns of the Brugada syndrome in right ventricular infarction/ischemia. Am J Cardiol. 2000; 86: 1056.
- ↑ 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) - ↑ 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) - ↑ Tomcsanyi J, Simor T, Papp L. Images in cardiology. Haemopericardium and Brugada-like ECG pattern in rheumatoid arthritis. Heart. 2002; 87: 234.
- ↑ Read DH, Harrington DD (1981). "Experimentally induced thiamine deficiency in beagle dogs: clinical observations". American Journal of Veterinary Research. 42 (6): 984–91. PMID 7197132. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ Sethi, Kamal K.; Sethi, Kabir; Chutani, Surendra K. (2014). "Early repolarisation and J wave syndromes". Indian Heart Journal. 66 (4): 443–452. doi:10.1016/j.ihj.2014.06.002. ISSN 0019-4832.