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Background  The condition of anomalous aortic origin of the right coronary artery (AAORCA) with an interarterial course leads to few, if any, clinical problems. Malignant presentation of the often non-significant AAORCA associated with conduction system abnormalities is a rare finding. Surgical repair, even for highly symptomatic patients, is still controversial. However, in this case, the surgery brought a paradigm shift in treatment modality, improving the symptoms of this patient.    Case presentation  We report a case of a 52-year-old man with severe chest pain and recurrent electrical storms with an implanted cardiac resynchronization therapy defibrillator (CRT-D) device. Coronary angiography and computed tomography (CT) revealed the rare anomalous aortic origin of the right coronary artery (AAORCA) with a high interarterial course between the aorta and the pulmonary trunk. As symptoms typically develop on exertion, placing the patient at an increased risk of ischemic distress, a stress myocardial perfusion imaging study helped to identify his high-risk status. Although patient-specific, a surgical repair was the only concrete step agreed upon after multiple collaborative discussions with the cardiac surgeons. Surgery significantly improved the symptoms, with the patient reporting resolution of his chest pain, as well as no documented inappropriate defibrillator activity on follow-up appointments.    Conclusion  One purpose of reporting the case study was to underscore the risk factors associated with AAORCA, challenging claims of its benign nature. This case complements existing findings demonstrating that ischemic distress consequent to the right coronary artery (RCA) compression may precede the rare incidence of an electrical storm. Importantly, the case-study emphasizes the significance of integrated multimodality imaging in clinical practice as well as providing real-world evidence for the efficacy of surgical repair in highly symptomatic patients with AAORCA with an interarterial course.

Anomalous right coronary artery with interarterial course depicting an unusual case of an electrical storm: a case presentation