Right single coronary artery as an incidental finding in Takotsubo syndrome and acute heart failure: Case report and review of the literature

Abstract Introduction Single coronary artery (SCA) with no associated congenital heart disease is a rare congenital anomaly. Most cases are asymptomatic and incidental findings, but SCA can cause ischemia, congestive heart failure, and sudden cardiac death (SCD). Case report A 44-year-old woman presented with Takotsubo cardiomyopathy and cardiogenic shock. Selective cannulation of the left coronary artery (LCA) was not possible on coronary angiography (CA); an SCA was revealed arising from the right sinus, continuing distally as the circumflex artery and thereafter as the left anterior descending artery. Coronary computed tomography angiography (CCTA) confirmed left main atresia and no coronary stenosis. Cardiac magnetic resonance imaging (MRI) showed diffuse myocardial edema and no perfusion defects. The patient's clinical course was favorable under conservative management. Discussion Our paper describes an incidental finding of right SCA. We report a Lipton type R-I, in which a dominant right SCA supplies the entire myocardium. It is the rarest SCA presentation, with an incidence of 0.0008%; only 15 cases have been reported in the literature, all of which were studied by CA. Of these 15, one had SCD, five angina, one ventricular arrhythmia and one complicated acute coronary syndrome. CCTA confirmed the diagnosis in seven patients, MRI in one and transesophageal echocardiography in another. Nine patients had coronary lesions. Two underwent coronary artery bypass grafting, one percutaneous intervention and 11 conservative treatment. Conclusion Right SCA with congenital absence of the LCA is one of the rarest coronary artery anomalies. In a significant percentage of patients it is associated with ischemia and can be life-threatening. CCTA and MRI are the modalities of choice for diagnosis and risk stratification.