Samir rafla. syncope in brugada syndrome


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Samir rafla. syncope in brugada syndrome

  1. 1. Comment on Syncope in Brugada syndrome Samir Morcos Rafla Emeritus professor of cardiology, Alexandria Univ. Previous chief of the cardiology dept. FACC, FESC, member EHRA Member steering committee of Egyptian Cardiac Arrhythmia Association ECRA
  2. 2. Patients initially presenting with aborted sudden death are at the highest risk for a recurrence (69%), whereas those presenting with syncope and a spontaneously appearing Brugada ECG sign have a recurrence rate of 19%. An 8% occurrence of cardiac events was observed in initially asymptomatic patients.
  3. 3. Among asymptomatic patients, those at highest risk displayed the Brugada sign spontaneously; those in whom ST segment elevation appeared only after provocation with sodium channel blockers appear to be at minimal or no risk for arrhythmic events.
  4. 4. 1 Patients have a high risk for sudden arrhythmic death, even in the absence of a history of prior cardiac arrest: 8.2% experienced sudden death or at least one documented episode of VF during a mean follow up of 24±33 months. A spontaneously abnormal Type I ECG carried a 7.7- fold higher risk of developing an arrhythmic event during a lifetime as compared to individuals in whom the ECG diagnostic of BS was evident only after sodium channel blocker challenge.
  5. 5. 2 Male gender is another risk factor for sudden death, because males had a 5.5-fold higher risk of sudden death as compared to females. 3 Programmed electrical stimulation resulting in inducibility of a sustained ventricular arrhythmia is the strongest marker of risk, associated with an 8-fold higher risk of (aborted) sudden death than non-inducible patients.
  6. 6. Inducibility of ventricular arrhythmias and a previous history of syncope are suggested to be markers of a poor prognosis in Brugada syndrome. Asymptomatic patients can be reassured if they are non-inducible.
  7. 7. Current recommendations for ICD implantation are summarized as follows: 1 Symptomatic patients displaying the Type 1 Brugada ECG (either spontaneously or after sodium channel blockade) who present with aborted sudden death should receive an ICD without additional need for EP study . Similar patients presenting with related symptoms such as syncope, seizure or noctural agonal respiration should also undergo ICD implantation after non-cardiac causes of these symptoms have been carefully ruled out.
  8. 8. 2 Asymptomatic patients displaying a Type 1 Brugada ECG (spontaneously or after sodium channel block) should undergo EPS if there is a family history of sudden cardiac death suspected to be due to Brugada syndrome. EPS may be justified when the family history is negative for sudden cardiac death if the Type 1 ECG occurs spontaneously. If inducible for ventricular arrhythmia, the patient should receive an ICD. Asymptomatic patients who have no family history and who develop a Type I ECG only after sodium channel blockade should be closely followed-up. Abnormal Normal
  9. 9. Brugada syndrome and ARVC/D show a different genetic background. The only gene thus far linked to the Brugada syndrome is the cardiac sodium channel gene which is the same gene implicated in the LQT3 form. Instead, ARVC/D has been linked to nine different chromosomal loci and three putative genes, which are unrelated to those discovered for Brugada
  10. 10. Conditions that can lead to ST segment elevation in the right precordial leads Right or left bundle branch block, left ventricular hypertrophy Acute myocardial ischemia or infarction Acute myocarditis Right ventricular ischemia or infarction, Prinzmetal’s angina Dissecting aortic aneurysm Acute pulmonary thromboemboli Various central and autonomic nervous system abnormalities Heterocyclic antidepressant overdose Duchenne muscular dystrophy, Friedreich’s ataxia Thiamine deficiency Hypercalcemia, Hyperkalemia Cocaine intoxication Mediastinal tumor compressing right ventricular outflow tract Arrhythmogenic right ventricular cardiomyopathy/dysplasia Long QT syndrome, type 3 Other conditions that can lead to ST segment elevation in the right precordial leads: Early repolarization syndrome Other normal variants (particularly in men)
  11. 11. Twelve lead ECG of a well-trained athlete without the Brugada syndrome