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An anomalous origin of the right coronary artery from the opposite sinus (R- ACAOS) with interarterial course is a very rare congenital anomaly with an increased risk of sudden cardiac death. A 29-year-old woman was admitted for exertional angina pectoris. A coronary computed tomography angiography with 3D multiplanar reconstruction revealed an R-ACAOS running between the aorta and pulmonary artery with high anatomical features and no ischemia-induced at the stress test. The heart team staff decided to manage the patient surgically. Coronary arteries are characterized by their origin, course, and boundaries with intracardiac structures and large vessels. Computed tomography angiography is the gold standard exam to confirm the diagnosis and identify high-risk anatomic features that guide the management. It is important to consider treatment options for these patients whether by medical treatment, coronary angioplasty, or surgical repair, because of the increased risk of SCD. We hereby present an R-ACAOS with interarterial course and high anatomic features, which is an uncommon finding and can be fatal if it remains undiscovered.

Abstract Background Bundle branch reentrant ventricular tachycardia is a unique type of VT that employs both bundles and the ventricular septum as integral components of a re-entrant circuit. It is usually observed in dilated cardiomyopathy, and its circuit depends exclusively on the specialized conduction system. Case presentation A 67-year-old man with a history of Fabry disease, and atrial fibrillation ablation, was admitted for a wide QRS tachycardia at 150 bpm, with an LBBB. Pharmacological therapy failed to stop the arrhythmia. Intracardiac recordings confirm the diagnosis. His Bundle recordings are essential, and Atrioventricular (AV) dissociation is typically present. The method used to induce left BBRVT is right ventricle apex stimulation. An electrophysiological study showed an AV dissociation a long HV sequence and a similar QRS morphology between VT and sinus rhythm. An overdrive stopped it. The same arrhythmia reappears and is restored to sinus rhythm by electrical cardioversion. We decided to implant an Implantable Cardiac defibrillator (ICD) with a backup pacing for secondary prevention and to ablate the right bundle branch. Conclusion BBRVT is a rare entity that is underreported. Pharmacological therapy is usually inefficient. An ICD is recommended for secondary prevention and can provide backup pacing, frequently required after catheter ablation to prevent the development of AV block or an excessive prolonged HV interval. Catheter ablation of the bundle branch is the first-line therapy.