Occlusion with a Vascular Plug of a Congenital Circumflex Coronary Artery—Right Ventricle Fistula with Coronary Steal Phenomenon

Background: Coronary fistulas are abnormal communications with a worldwide prevalence of 0.002% in the general population. Aim: The aim of this study is to present a case of a congenital coronary artery fistula presented with coronary steal and describe this phenomenon. Methods: A systematic search was conducted to explore the relationship between the coronary steal phenomenon and coronary artery diseases using the PubMed database. Case Presentation: A seven-month-old female patient, was admitted to the hospital presenting diaphoresis on feeding and failure to thrive. Physical examination denotes continuous murmur in the fourth intercostal space left parasternal border (IV/VI) crescendo-decrescendo, wide and permanent unfolding of S2 (S1-A2-P2). An echocardiogram shows enlargement of right cavities, dilatation of the left coronary artery, circumflex artery connected to the right ventricle, and pulmonary artery systolic pressure of 45 mmHg. Tomography angiography demonstrated dilatation of the circumflex artery and a tortuous trajectory towards the posterior wall of the right ventricle, suggesting a coronary fistula. Results: The findings were corroborated by catheterization and a vascular plug (Amplatzer AVP II) was successfully placed. During follow-up complete obliteration was observed, with adequate weight gain and no associated symptoms. Conclusion: This case illustrates a distinctive clinical manifestation of congenital coronary artery fistula associated with the coronary steal phenomenon, establishing a new paradigm for the early diagnosis and management of this condition in the pediatric population.