Brugada syndrome historical perspective

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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 was potentially first seen on EKG in survivors of cardiac arrest in 1989, but it was not until 1992 that the Brugada brothers recognized it as a distinct clinical entity which could cause sudden death by ventricular fibrillation.

Discovery

  • Sudden unexplained death syndrome was first noted in 1977 by Hmong refugees in the US.[1][2]
  • The disease was again noted in Singapore, when a retrospective survey of records showed that 230 otherwise healthy Thai men died suddenly of unexplained causes between 1982 and 1990: publication of this data provoked a diplomatic incident.[3]
  • The condition appears to affect primarily young Hmong men from Laos (median age 33) and northeastern Thailand (where the population are mainly of Laotian descent).[4][5]
  • There is a strong hereditary component and the victims tend to die in their sleep. Survivors describe a feeling of intense fear and paralysis. There is a sensation of pressure in the chest, the presence of an alien being in the room and altered sensation.[6]
  • This phenomenon is well known among the Hmong people of Laos, who ascribe these deaths to a malign spirit, dab tsog (pronounced "da cho"), said to take the form of a jealous woman. Hmong men may even go to sleep dressed as women so as to avoid the attentions of this spirit.[7]
  • The Brugada brothers were the first to describe the characteristic ECG findings and link them to sudden death. Before that the characteristic ECG findings were often mistaken for a right ventricular myocardial infarction. In 1953 a publication by Oscher mentioned that despite being mistaken for right ventricular myocardial infarction, the ECG findings were not associated with myocardial ischemia.[8]
  • Although the ECG findings of Brugada syndrome were first reported among survivors of cardiac arrest in 1989, it was only in 1992 that the Brugada brothers recognized it as a distinct clinical entity, causing sudden death by causing ventricular fibrillation.[9][10]

References

  1. Centers for Disease Control (CDC) (1981). "Sudden, unexpected, nocturnal deaths among Southeast Asian refugees". 30 (47): 581&ndash, 4, 589. PMID 6796814. Text "MMWR Morb Mortal Wkly Rep" ignored (help)
  2. Parrish RG, Tucker M, Ing R, Encarnacion C, Eberhardt M (1987). "Sudden unexplained death syndrome in Southeast Asian refugees: a review of CDC surveillance". MMWR CDC Surveill Summ. 36 (1): 43SS–53SS. PMID 3110586.
  3. Goh KT, Chao TC, Chew CH (1990). "Sudden nocturnal deaths among Thai construction workers in Singapore". Lancet. 335: 1154. PMID 1971883.
  4. Tatsanavivat P, Chiravatkul A, Klungboonkrong V, Chaisiri S, Jarerntanyaruk L, Munger RG, Saowakontha S (1992). "Sudden and unexplained deaths in sleep (Laitai) of young men in rural northeastern Thailand". Int J Epidemiol. 21 (5): 904&ndash, 10. PMID 1468851.
  5. Tungsanga K, Sriboonlue P (1993). "Sudden unexplained death syndrome in north-east Thailand". Int J Epidemiol. 22 (1): 81&ndash, 7. PMID 8449651.
  6. Shkolnikova, M.A. Shkolnikova; Miklashevich, I.M. Miklashevich (2016). "The Brugada Syndrome as a Cause of Sudden Death. Diagnostics and Clinical Manifestations in Children". Kardiologiia. 10_2016: 63–71. doi:10.18565/cardio.2016.10.63-71. ISSN 0022-9040.
  7. Adler SR (1995). "Refugee stress and folk belief: Hmong sudden deaths". Soc Sci Med. 40 (12): 1623&ndash, 9. PMID 7660175.
  8. OSHER HL, WOLFF L (1953). "Electrocardiographic pattern simulating acute myocardial injury". The American Journal of the Medical Sciences. 226 (5): 541–5. PMID 13104407. Unknown parameter |month= ignored (help); |access-date= requires |url= (help)
  9. Martini B, Nava A, Thiene G, Buja GF, Canciani B, Scognamiglio R, Daliento L, Dalla Volta S. Ventricular fibrillation without apparent heart disease: description of six cases. Am Heart J 1989 Dec;118(6):1203-9 PMID 2589161
  10. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol. 1992 Nov 15;20(6):1391-6. PMID 1309182


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