Interrupted Aortic Arch in an Adult

Eur J Case Rep Intern Med. 2017 Aug 3;4(9):000692. doi: 10.12890/2017_000692. eCollection 2017.

Abstract

Interrupted aortic arch (IAA) is a rare congenital abnormality with only a few cases reported in adults. It is defined as complete loss of continuity between the ascending and descending portions of the aorta, and is usually associated with other cardiac defects. The diagnosis in adults should be suspected in the presence of refractory hypertension, a careful physical examination being crucial to early diagnosis. Magnetic resonance angiography (MRA) techniques can accurately characterize cardiovascular anatomy, and also provide information regarding heart chamber and valve function.

Learning points: Although rare, interrupted aortic arch (IAA) must be considered in the differential diagnosis of adults with refractory hypertension. A careful physical examination, with evaluation of femoral pulses and blood pressure in the four limbs, is essential for the diagnosis and for ensuring that the correct diagnosis is made promptly.This case highlights the value of magnetic resonance angiography (MRA) in radiation-free non-invasive evaluation of adults with congenital aortic abnormalities, as it may provide clinically important haemodynamic information.Only a few cases have been reported in adults. We present an unusual case in which the diagnosis was not made until adulthood.

Keywords: Interrupted aortic arch; aortic coarctation; bicuspid aortic valve; refractory hypertension.