We report a 1-month-old infant diagnosed with an aorta–left ventricular tunnel, ventricular septal defect, and right coronary atresia with right ventricular sinusoids. The patient’s anatomy and physiology did not indicate right-ventricular-dependent coronary circulation, and therefore right ventricular decompression could be performed without compromising coronary perfusion during surgical correction. A detailed understanding of the coronary anatomy is critical in managing this defect when coronary anomalies are present.
