Brugada Syndrome

Brugada Syndrome is an ECG abnormality with a high incidence of sudden death in patients with structurally normal hearts.

First described in 1992 by the Brugada brothers, the disease has since had an exponential rise in the numbers of cases reported. The mean age of sudden death is 41, with the age at diagnosis ranging from 2 days to 84 years. It has been previously described as Sudden Unexplained Nocturnal Death Syndrome (SUNDS). 

Brugada Syndrome Key Points

  • There’s really only one type of Brugada syndrome.
  • Diagnosis depends on a characteristic ECG finding AND clinical criteria.
  • Definitive treatment = ICD.
  • Brugada sign in isolation is of questionable significance.

Diagnostic Criteria

Type 1
  • Coved ST segment elevation >2mm in >1 of V1-V3 followed by a negative T wave.
  • This is the only ECG abnormality that is potentially diagnostic.
  • It is often referred to as Brugada sign.
  • Documented ventricular fibrillation (VF) or polymorphic ventricular tachycardia (VT).
  • Family history of sudden cardiac death at <45 years old .
  • Coved-type ECGs in family members.
  • Inducibility of VT with programmed electrical stimulation .
  • Syncope.
  • Nocturnal agonal respiration.

Management

The only proven therapy is an implantable cardioverter – defibrillator (ICD).

Undiagnosed, Brugada syndrome has been estimated to have a mortality of 10% per year. Does this mean that a diagnosis in ED mandates admission? Probably yes for all type 1 patients if they present with suggestive clinical criteria.

Source: https://litfl.com/brugada-syndrome-ecg-library/

Further reading: https://emedicine.medscape.com/article/163751-overview


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