Risk management in early stage arrhythmogenic cardiomyopathy associated with Provocable Brugada ECG

Risk factors of malignant tachyarrythmias should be assessed in cases with early stage arrhythmogenic cardiomyopathy and provocable Brugada ECG. Spontaneous right precordial coved-type ST segment elevation, tall R wave in lead aVR, QRS fragmentation, 1° AV block, inducibility during electrophysiological examination, low amplitude coved-type ST elevation and localised right precordial QRS prolongation should be tested in 20 out of 128 patients. Only QRS fragmentation and localised right precordial QRS prolongation were positive in these patients. QRS fragmentation was positive in two patients, one with aborted sudden cardiac and one patient without any further arrhythmic events. Localised right precordial QRS prolongation was positive in five patients with serious arrhythmic events – four cases with previous ventricular fibrillation and one case with subsequent slow ventricular tachycardia. All other risk factors could be ruled out. In summary, localised right precordial QRS prolongation seems to be the strongest risk marker in cases of developing arrhythmogenic cardiomyopathy and provocable Brugada ECG