This review focuses on a rare complication of pulmonary arterial hypertension (PAH), extravasation compression of the left coronary artery (LCA) dilated by the pulmonary artery. The review described clinical manifestations and methods for diagnostics of LCA compression, and advantages of the endovascular correction of this complication in patients with pulmonary hypertension. Selection of a device to be implanted during the endovascular intervention in these patients was discussed with due account for concomitant treatment with oral anticoagulants. As an illustration of the issue under discussion, a clinical case of acute coronary syndrome in a female patient from the PAH Registry of the V. A. Almazov National Medical Research Center was provided.