Long-Term Electrocardiographic Follow-up From Childhood of an Adult Patient With Brugada Syndrome Associated With Sick Sinus Syndrome

A Case Report

Abstract

We had the unique opportunity of following the electrocardiographic (ECG) course of a 13-year-old male with sinus dysfunction and atrial flutter who subsequently developed a Brugada-type ECG pattern associated with sick sinus syndrome at 25 years old. Family history showed that the patient's mother and maternal grandfather suddenly died while sleeping at night. When the patient was 13 years old, he lost consciousness after running a marathon. The patient was diagnosed with sinus dysfunction and atrial flutter, and he underwent pacemaker implantation at 15 years old. ECG examinations performed between 13 and 20 years old showed incomplete right bundle branch block and ST elevation with early depolarization. On ECG examinations performed when the patient was 21 years old and thereafter, the V₂ lead always showed a saddleback-type ST elevation. At 25 years old, the late potential was positive and the electrophysiological study induced ventricular fibrillation. A challenge test with pilsicainide showed remarkable ST elevation by the V₂ lead. The 24-h Holter ECG monitoring showed remarkable ST elevation after eating a snack and during night time when the patient was asleep. The patient was diagnosed with Brugada syndrome and an implantable cardioverter-defibrillator was implanted. Genetic analysis did not reveal mutation of the SCN5A gene. (Circ J 2009; 73: 575 - 579)