Should This Man be Worried About Sudden Death?

ANSWER

The ECG shows normal sinus rhythm, with a right-axis deviation (R wave axis > 90°) and a significant ST elevation noted in the anterior precordial leads consistent with a type 1 Brugada pattern. In this ECG, the elevated ST segment (≥ 2 mm) descends into an inverted T wave in leads V1 and V2. 

The Brugada syndrome is due to a mutation in SCN5A, the alpha subunit of the cardiac sodium channel gene. Any clinical expression of the Brugada syndrome is related to ventricular arrhythmias, which are life-threatening.

Given his family history and his ECG, the patient is correct to be concerned. Genetic testing should be performed, and he should be referred for evaluation, including placement of an implantable defibrillator system for primary prevention of sudden cardiac death.