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October 2007

By Shefali Gupta, MD, Vivek Mangla, MD, FACC, FSCAI, Aditya Gupta, MD, and John E. Francis, MD

Clinical Presentation

A 42-year-old man presents to your emergency department with frequent episodes of palpitations and dizziness accompanied by shortness of breath. These episodes last 5 to 10 minutes, occur three to four times a day, and have persisted for the last five months. The patient had a single episode of syncope about two months ago for which he did not seek medical treatment. He takes amlodipine and benazepril for hypertension. Serial cardiac enzymes are negative. A two-dimensional echocardiogram and a coronary angiogram are within normal limits. This ECG is obtained.

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diagnosis and discussion

The initial ECG reveals a normal sinus rhythm of 75 bpm. Leads V1 and V2 reveal an incomplete right bundle branch block pattern with accompanying ST-segment coving, especially in lead V2. This is characteristic of type I Brugada syndrome. Polymorphic ventricular tachycardia was induced on an electrophysiologic study (below). An automated implantable cardioverter-defibrillator was placed, and the patient was asymptomatic at his three-month follow-up visit.

Patients with Brugada syndrome may have spontaneous episodes of polymorphic ventricular tachycardia or ventricular fibrillation. When these episodes terminate spontaneously, the patient develops syncope. When the episodes are sustained, full-blown cardiac arrest and, eventually, sudden death occur. Approximately 60% of patients with sudden death and the typical Brugada syndrome ECG have a family history of sudden death or have family members with the same ECG abnormalities. Brugada syndrome is more prevalent in men.

Sometimes this ECG pattern is misinterpreted as an acute ST-elevation myocardial infarction. Proper ECG recognition is especially important in individuals with Brugada syndrome since the first presentation may be sudden cardiac death.

 

 




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