Takayasu’s arteritis and fibromuscular dysplasia as causes of acquired atypical coarctation of the aorta: retrospective analysis of seven cases

Abstract

Coarctation of the aorta usually occurs in the aortic isthmus: however, 2% of all coarctation is localized at atypical sites such as the aortic arch and the thoracic and/or abdominal aorta. Causal therapy involves vascular surgery, and during the procedure, biopsies of the involved vessels should be taken to establish the etiology. This study involved the retrospective analysis of clinical and histopathological findings for seven patients who underwent vascular surgical procedures (age range, 10-37 years; male/female ratio 3:4). Histopathological analysis of specimens revealed two pathological processes taking place with different localization in the aortic wall as the cause of the atypical coarctation of the aorta: (1) fibrosis/scarification in the tunica media and adventitia compatible with chronic lesions of Takayasu's arteritis (four cases); (2) fibromuscular dysplasia in the tunica media (three cases).

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

“…Arterial stenoses are commonly present in Takayasu arteritis, giant-cell arteritis, and middle aortic syndrome, although these are inflammatory lesions that identify an origin distinct from FMD. [119][120][121][122] Aneurysms may be present in these diseases, and the stenoses are commonly tubular or focal in nature. The arteriographic appearance of arterial segments demonstrating long areas of smooth narrowing is classic for vasculitis but not pathognomonic.…”

Surgical treatment of atypical aortic coarctation complicating Takayasu’s arteritis—experience with 33 cases over 44 years

“…Aortic coarctation is typically located either proximal or distal to the ligamentum arteriosum in the aortic isthmus, whereas atypical aortic coarctation (AAC) has been reported to occur anywhere along the length of the aorta except for the ascending aorta. 1,2 AAC is a rare condition associated with Takayasu's arteritis (TA), fibromuscular dysplasia, congenital hypoplasia, and atherosclerosis, [2][3][4] and is characterized by symptoms caused either by secondary hypertension in the upper half of the body or hypotension in the lower extremities.…”

Thoracoabdominal aorta coarctation with bilateral renal artery involvement: diagnosis with multidetector CT angiography (MDCTA)

“…AAC is a rare condition that accounts for less than 2% of aortic coarctation. When longer segments of the aorta are narrowed, the term hypoplasia was used by some authors [6,7].…”