ABSTRACT
<p><b>OBJECTIVE</b>to investigate the clinical characteristics in two families with early repolarization syndrome (ERS) and recurrent syncope.</p><p><b>METHOD</b>all family members including the probands were screened with routine clinical examination, electrocardiography, echocardiography, Holter recording, chest x-ray, head-up tilt test and blood biochemistry.</p><p><b>RESULTS</b>there was no clinical evidence of organic heart disease in all members from the two families. Proband 1 showed recurrent syncope, ERS and repeated torsade de pointes ventricular tachycardia and ventricular fibrillation were documented with resting ECG. ERS was detected in one brother, one nephew and one son from him and all were free of cardiac events including syncope, cardiac arrest and sudden cardiac death. Proband 2 showed recurrent syncope, ERS and ST segment arched upward elevation in V(1)-V(3) were documented by ECG. His father suffered sudden cardiac death at the age of 65 and asymptomatic ERS was detected in one of his nephew.</p><p><b>CONCLUSIONS</b>ERS is not always linked with benign clinical course and can sometimes lead to repeated syncope, torsade de pointes ventricular tachycardia and ventricular fibrillation. Pedigree research is of importance for ERS.</p>