Brugada Syndrome and Right Ventricle Morphofunctional Abnormalities on Echocardiography in Young Male with Family Anamnesis of Sudden Cardiac Death

Robert Steiner, Sandra Makarovic, Zorin Makarovic, Ines Bilic-Curcic

Abstract


First presented by Brugada and Brugada in 1992, Brugada Syndrome (BrS) is a primary electrical disease of the heart that causes sudden cardiac death or life-threatening ventricular arrhythmias. This disease is hereditary autosomic dominant transmitted and genetically determined. The syndrome has been linked to mutationsin SCN5A, the gene encoding for the α-subunit of the sodium channel. Electrocardiogram (ECG) abnormalities indicating Brugada syndrome, include repolarization and depolarization abnormalitiesin the absence of identifiable structural cardiac abnormalitiesor other conditions or agents known to lead to ST-segment elevationin the right precordial leads (V1-V3). Intravenous administration of sodium channel blocking drugs may modify the ECGpattern. Ajmaline, flecainide, procainamide and propafenone exaggerate the ST-segment elevation or unmaskit when it is initially absent. An implantable cardioverter-defibrillator (ICD) is the only proven effective device treatment for the disease. Although BrS is primary electrical disease, some authors have suggested the presence of morphological and functional abnormalities mainly located in the right ventricle (RV), notably in the outflow tract (RVOT). In this short report we will present a young male, with predisposition and positive family history of sudden cardiac death, with complete diagnostic procedure including propafenon testing unmasking Brugada syndrome. An echosonography revealed dilated apical right ventricle, suggesting BrS is not only electrical disorder, but may include morphofunctional abnormalities, described in previous reports. In addition, we reviewed the possible connection between Brugada syndrome and morphological abnormalities in RV.


Keywords*


Brugada syndrome, sudden cardiac death, electrical disorder, sodium chanel blocking agents, inheritance, propafenon test, morphological abnormalities in RV, implantable cardioverter defibrillator, arrhytmogenic right ventricular cardiomyopathy, ST-segment

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