1113 - Cardiac arrest in a 21-year-old due to rare congenital anatomic variant with superimposed Brugada type I

A 21-year-old male with one-year history of presyncope presented after sudden collapse while playing basketball due to cardiac arrest. Prolonged PR interval observed on resting electrocardiogram seven months prior. Cardiac imaging revealed anomalous right coronary artery (ARCA), with the right coronary artery originating from the left coronary cusp, leading to severe origin site stenosis and cardiac ischemia. Cardiogenetic testing revealed SCN5A loss of function mutation, indicating Brugada syndrome type I. Inpatient rehabilitation necessitated wearable cardioverter-defibrillator; course initially notable for impaired memory, inattention, delayed processing, and decreased activity tolerance as well as impaired static and dynamic balance with truncal instability and scissoring gait pattern. Surgical ARCA unroofing with implantable cardioverter-defibrillator is planned.

Discussions:

Coronary artery anomalies (CAA) are congenital disorders involving an abnormal origin or course of any of the three main epicardial coronary arteries. CAA affect 0.2–5.8% of the population and are highly associated with exercise-induced sudden cardiac death. ARCA describes a rare CAA variant wherein the right coronary artery originates from a site other than the right aortic sinus. As seen here, ARCA can lead to clinically significant vessel caliber reduction during vigorous activity and cardiac ischemia. Brugada syndrome causes cardiac conduction abnormalities predisposing to ventricular arrhythmias and sudden death. 

Cardiac rehabilitation in congenital heart disease is a relatively new and controversial field, with most data centered on the post-surgical period. Few cases of ARCA with Brugada syndrome exist, and no guidelines or safety data on cardiac rehabilitation prior to surgical correction are described in the literature.

Conclusions: The co-occurrence of ARCA and Brugada syndrome predisposes to sudden cardiac death and presents a unique rehabilitative challenge, particularly prior to surgical correction. Few guidelines exist regarding preoperative cardiac rehabilitation in this population as well as for other types of CAA, warranting further study.