1255 Previously unknown ventricular arrhythmia in a globally delayed pediatric patient with tuberous sclerosis

Abstract

Abstract Introduction We present a case of previously unknown ventricular ectopy in a pediatric patient who is globally delayed. As the patient is non-verbal, it is possible the patient may have ultimately experienced heart failure or a lethal arrhythmia were it not for the polysomnogram (PSG). Report of Case A four year old female with Tuberous Sclerosis (TS) was referred to our pediatric sleep center for snoring. Her past medical history includes intracardiac tumors, daily seizures, and global developmental delay. Initial PSG showed moderate obstructive sleep apnea defined by an obstructive Apnea-Hypopnea Index of 6.89 and no cardiac arrhythmias. Positive airway pressure titration study was performed one year later. Premature ventricular contractions were noticed during the setup while patient was awake. Intermittent couplets and triplets occurred during sleep as well as a prolonged run of bigeminy that initiated during a period of wake after sleep onset and persisted into non-rapid eye movement sleep. She was escorted to the emergency room where a 12-lead electrocardiogram (EKG) showed sinus rhythm with non-specific interventricular conduction delay and right ventricular hypertrophy. She was discharged with a Holter monitor and subsequent analyses was concerning for frequent ventricular couplets and non-sustained runs of ventricular tachycardia. She required admission to initiate anti-arrhythmic therapy. Imaging revealed stable intracardiac tumors, but revealed scarring within sites of intramyocardial lesions. She failed different anti-arrhythmic agents before settling with amiodarone. Conclusion Sleep disordered breathing (SDB) can cause or worsen cardiac arrhythmias. This case highlights the importance of routine surveillance in patients with both known cardiac disease who have or are at risk of having significant cardiac arrhythmias and suspected SDB. Additionally, current guidelines for TS patients recommend EKG once every 3 to 5 years. This case may also highlight the importance of increased cardiac surveillance in this population group.