BACKGROUND: Interrupted aorta is a rare congenital malformation defined as the lack of continuity between the ascending and descending parts of the aorta.

CASE REPORT: This malformation was first described by Steidele in 1778. To date a few isolated adult interrupted aortic arch patients have been reported and most of them were treated surgically. However, there is not data about outcome of patients who decline surgery or who are not good candidates for surgery because of excessive risks, and there is not a data about how to follow these patients.

CONCLUSIONS: Herein we present a case of adult type A isolated interrupted aorta followed-up for 4 years by medical therapy without complications.

Keywords: Angiotensin II Type 1 Receptor Blockers - therapeutic use, Angiography, Aorta, Thoracic - radiography, Biphenyl Compounds - therapeutic use, Blood Flow Velocity, Blood Pressure - drug effects, Calcium Channel Blockers - therapeutic use, Dihydropyridines - therapeutic use, Diuretics - therapeutic use, Drug Therapy, Combination, Follow-Up Studies, Hydrochlorothiazide - therapeutic use, multidetector computed tomography, Tetrazoles - therapeutic use, Time Factors, Vascular Malformations - radiography