B-ab19-02 surgical ablation of right ventricular arrhythmic substrate as a consequence of anomalous right coronary artery

Coronary artery anomalies have been reported as a frequent cause of sudden cardiac death in young athletes following cardiomyopathies and channelopathies. To recognize anomalous coronary artery as a cause of sudden cardiac arrest in a structurally normal heart. N/A A 40-year-old endurance athlete with history of recurrent exertional syncope presented with wide complex tachycardia. Baseline ECG was within normal limits. An echocardiogram revealed normal biventricular function. A cardiac MRI demonstrated inferior right ventricular (RV) epicardial scarring. EP study showed easily inducible sustained monomorphic ventricular tachycardia (VT) with left bundle branch morphology and left superior axis. An anomalous right coronary artery was revealed by a CT-angiogram with slit-like origin above the commissure of the left and right aortic cusps, coursing between the aorta and the pulmonary artery. Cardiopulmonary bypass was performed, with marsupialization of the anomalous right coronary artery followed by epicardial mapping. Multiple late fractionated signals were mapped to a pale area overlying the inferior RV and cryoablation lesions were delivered. On repeat EP study, VT was no longer inducible despite aggressive programmed stimulation. Implantable cardiac monitor demonstrated no further arrhythmic events during one year follow up. Repeated ischemic insults from anomalous right coronary artery can lead to myocardial fibrosis and the generation of arrhythmic substrate. Recognition and management of this substrate with ablation and correction of the inciting coronary anomaly can prevent malignant cardiac arrhythmia.