The Beat Webinar Series - Episode 8 - LIVE from New Orleans, Cases of Genetic Arrhythmias (On Demand)-The Beat Episode 8

Video Transcription

Video Summary

The panel discussed three cases of arrhythmias in young patients. The first case involved a boy who experienced recurrent ventricular tachycardia (VT) and ventricular fibrillation (VF) due to catecholaminergic polymorphic ventricular tachycardia (CPVT). Genetic testing initially came back negative, but later revealed loss of function mutations in triadine, a gene responsible for normal cardiac contraction. The boy was treated with nadolol, esmolol, and flecainide, and underwent left cardiac sympathetic denervation. The second case involved a newborn with bradycardia and long QT syndrome (LQTS). The baby's heart rate was initially below the third percentile, but later showed sinus rhythm with 2:1 AV conduction and severe QT prolongation. Genetic testing revealed a variant of uncertain significance in calmodulin 2, a gene involved in inactivation of the L-type calcium channel. The baby was treated with lidocaine, esmolol, and later propranolol and flecainide. The third case involved a four-year-old girl with frequent VT/VF episodes. The girl was diagnosed with Brugada syndrome, but genetic testing initially did not identify a mutation. Treatment with flecainide and sotalol was successful in controlling her arrhythmias, and she underwent focal atrial tachycardia ablation. The panel emphasized the need for continued monitoring and aggressive treatment of fever in these patients.

Keywords

arrhythmias

young patients

ventricular tachycardia

genetic testing

long QT syndrome

Brugada syndrome

fever monitoring