BackgroundIsolated congenital ostial stenosis of the left coronary artery (LCA) is extremely rare, and available literature is limited. Long-term treatment success is key in the choice of treatment strategy due to the mostly young age of the patients. Here, we present a clinical case and shed light on the surgical treatment strategies including their pitfalls.Case presentationWe describe a 20-year-old male who presented to the emergency department with recurrent typical angina pectoris symptoms (CCS class II). Computed tomography and coronary angiography revealed isolated ostial stenosis of the LCA, with prominent collaterals from the right coronary artery. The patient was operated on and intra-operative findings showed a severely narrowed LCA ostium that appeared to be fibrotic, and to originate from a similarly fibrotic left coronary sinus of the aortic root. The LCA was excised from the left coronary sinus, and trimmed until the coronary artery lumen appeared macroscopically normal. The fibrotic left coronary sinus was resected and replaced with a bovine pericardial patch, into which the coronary artery was re-inserted. During weaning from cardiopulmonary bypass, we faced a diminished left ventricular function. We attributed this to insufficient myocardial protection. Isolated antegrade cardioplegia had been used, which, for technical reasons, had to be administered separately to the right and left coronary artery after aortotomy. In the setting of huge collaterals from the right to the left coronary artery, a steal-effect likely occurred. After prolonged reperfusion, the left ventricular function recovered, and the further post-operative course was unremarkable.ConclusionsFor the surgical treatment of congenital ostial stenosis of the LCA, both ostium reconstruction and coronary artery bypass grafting have been described. The advantage of ostium reconstruction as chosen here is to create a physiological flow and supply situation to the affected myocardial areas. Furthermore, in case of other downstream events, such as the development of coronary artery disease, all further therapeutic options are preserved.Special attention should be paid to the administration of cardioplegia in these patients. Combined ante and retrograde cardioplegia administration probably would have achieved more extensive myocardial protection in our case.