PO-01-085 ATRIAL AND VENTRICULAR ARRHYTHMIAS IN A PATIENT WITH TUBEROUS SCLEROSIS

Abstract

Tuberous Sclerosis is a genetic condition characterized with multiple non-cancerous tumor growths throughout the body. While arrhythmias are commonly seen in these patients, the predominance of arrhythmias appear to be irregular with atrial and ventricular ectopy as the most common manifestation. In our case we describe a patient with both organized atrial arrhythmias ( typical atrial flutter, atypical atrial flutter ) and monomorphic ventricular tachycardia. A pleasant 22 year old female with a pertinent medical history of Tuberous Sclerosis with multiple tubers present in the heart and brain presented to the electrophysiology clinic for evaluation regarding palpitations. The patient had a known history of prior ablation for typical atrial flutter twice before with recurrence of arrhythmias despite ablation and long term anti-arrhythmic therapy with flecainide. Implantation of an implantable loop recorder revealed evidence of likely typical flutter as well as monomorphic ventricular tachycardia. During the electrophysiology study, typical atrial flutter and atypical atrial flutter was succesfully induced and ablated. No ventricular arrhythmias could be induced. In addition intracardiac echocardiography revealed the presence of rhabdomyomas in the cavo-tricuspid isthmus and right side of the interventricular septum. NA Ablation across the CTI and a posterior line from the SVC to IVC resulted in tachycardia termination with no further induction of atrial arrhythmias and the presence of bidirectional block. No ventricular arrhythmias could be induced. Arrhythmias are a common manifestation in Tuberous Sclerosis. Our case highlights the arrhythmogenic nature of rhabdomyomas that can be present in both the atrium and ventricle. There is a scarcity of data available regarding the overall burden of arrhythmias in this patient population. In addition our patient is unique in that they presented with organized atrial and ventricular arrhythmias. The mechanism of the ventricular arrhythmia is unclear as it was not able to be induced, however, both atrial arrhythmias appeared macro-reentrant. While some histopathologic studies have suggested the presence of macro-renetrant circuits in this patient population, further large scale studies would be helpful to help characterize the mechanism of arrhythmias in this patient population.