Subclavian steal syndrome associated with the right aortic arch in a patient with Peutz-Jeghers syndrome: case report

We report an adult case who presented vertigo attacks due to subclavian steal syndrome associated with the right aortic arch in a patient with Peutz-Jeghers syndrome. A 29-year-old male diagnosed as having Peutz-Jeghers syndrome developed frequent vertigo attacks and was admitted to our hospital. Blood pressure of the left arm was 20 mmHg lower than that of the right arm. Aortagraphy showed that he had a right aortic arch and isolation of the left subclavian artery. Right vertebral angiography opacified the left vertebral artery and the subclavian artery in retrograde fashion, suggesting subclavian steal phenomenon. Blood flow studies disclosed impaired reactivity to acetazolamide in the bilateral cerebellar hemispheres. We successfully carried out left common carotid artery-transverse cervical artery bypass. Postoperative angiography confirmed the patency of the graft and the disappearance of subclavian steal phenomenon. Vasoreactivity to acetazolamide normalized in the cerebellum. Vertigo attacks were not noted during the one-year follow-up period.