Brugada Syndrome

The BMJ 'Picture Quiz' this week includes an interesting case scenario:

A young man is admitted to CCU with central chest pain which occurred two hours after an episode of epigastric pain. The chest pain was associated with vomiting, dizziness, shortness of breath and sweating but the pain resolved prior to hospital admission. Twelve lead ECG shows ST elevation that gradually descends into inverted T waves in V1/V2. Echocardiogram was normal. Later in the day, he becomes nauseated and pale with a transient episode of blurred vision, muffled hearing and paraesthesia but no chest pain on this occasion. His blood pressure drops to 89mmHg and the heart monitor shows a pause of 3 seconds before return of QRS complexes. He has recently been suffering from flu-like symptoms and has previously had episodes of syncope in the past. 

Brugada syndrome is an autosomal dominant inherited condition characterised by distinctive ECG changes causing arrhythmias, syncope and risk of sudden death due to ventricular arrhythmias. Clinical symptoms usually start in the 3rd / 4th decade of life and often occur during sleep. Diagnosis is based on ECG findings and clinical symptoms. Physical examination should be performed to rule out any other potential cause of syncope and imaging (ECHO) is required to rule out structural causes which may explain the patient's symptoms. An implanted cardioverter defibrillator is the definitive treatment for patients at risk. Advice for patients with Brugada syndrome is to avoid the development of a high fever and any arrythmogenic drugs. Electrolyte disturbances may also precipitate development of an arrythmia in these cases.