Middle Aortic Syndrome: From Presentation to Contemporary Open Surgical and Endovascular Treatment

Abstract

Middle aortic syndrome (MAS) is a clinical condition generated by segmental narrowing of the abdominal or distal descending thoracic aorta. MAS may be acquired, caused by Takayasu's or temporal arteritis (giant cell arteritides), neurofibromatosis, fibromuscular dysplasia, retroperitoneal fibrosis, mucopolysaccharidosis, and the Williams syndrome, or congenital, ascribed to a developmental anomaly in the fusion and maturation of the paired embryonic dorsal aortas. Segmental aortic stenosis may be located at the suprarenal, inter-renal or infrarenal aorta, with a high propensity for concomitant stenoses in both the renal (63%) and visceral (33%) arteries. Hypertension proximal to the aortic stenosis, and relative hypotension distal to it, are characteristic findings in MAS. Typical manifestations include headache, early fatigue on exertion, and bilateral lower-limb claudication. The severity of hypertension is the primary indication for intervention and the factor determining procedural timing. As a great proportion of patients with MAS are children or teenagers, the clinical benefits of early surgical intervention to reverse refractory hypertension have to be weighed against the repercussions pertaining to the insult of surgery on the developing aorta. Open surgery is the primary treatment of tubular aortic narrowing (MAS) associated with renovascular hypertension and visceral artery stenosis. This entails aortoaortic bypass of the diseased segment or, less often, patch aortoplasty and usually bypass grafting of the stenosed renal and visceral arteries performed with autologous conduits, particularly in the youngest of patients. Endovascular therapy may provide a sound minimally invasive treatment in MAS caused by discrete aortic stenoses that do not encompass the mesenteric and renal arteries. Hypertension is thus improved or cured in more than 70% of patients. Prognosis after uncompromised surgical reconstruction is rewarding in the mid and long term in patients with congenital aortic coarctation but deteriorates in patients with aortoarteritis and recurrent inflammatory activity.

Open Surgical Treatment of a Severe Case of Obstructive Calcifying Aortic Disease

“…Many anatomic and morphologic patterns have been described for this syndrome. 2 Although the calcific component was suggestive of atherosclerotic degeneration, the descending thoracic aorta was the only vessel involved, whereas the splanchnic arteries, the infrarenal aorta, the iliac arteries, the supra-aortic trunks, and the coronary arteries all appeared normal. We searched the literature to better identify this clinical case.…”

“…1 It may be caused by congenital or inflammatory disorders, whose commonest disease is the Takayasu arteritis. 2 A similar nosologic entity is the ''coral reef aorta,'' which however involves the portion between the diaphragm and the suprarenal part of this vessel (visceral aorta). 3 Mild stenosis may be completely asymptomatic; with the progression of the disease, symptoms may arise, such as dyspnea, lipothymic episodes, fatigue, headache, upper limb hypertension, and lower limb hypoperfusion with claudication.…”

Analysis of Intraorganic Arteries and Renal Corpuscles in Chronic Hypotension

“…Изучение почек при коарктации аорты вызывает повышенный интерес у клиници-стов [14,16]. При постстенотической гипотен-зии у больных развивается сопутствующий стеноз почечных артерий [14,15], ишемия по-чек [15], появляются признаки хронической почечной недостаточности [17].…”

“…При постстенотической гипотен-зии у больных развивается сопутствующий стеноз почечных артерий [14,15], ишемия по-чек [15], появляются признаки хронической почечной недостаточности [17]. Сведения о функциональном состоянии почек у больных коарктацией аорты весьма противоречивы: одни исследователи указывают на его грубые нарушения другие -не отмечают выражен-ных расстройств [12].…”

Mid-aortic syndrome in two preterm infants

“…1 It is characterized by segmental narrowing of the distal thoracic or abdominal aorta and stenosis of one or both renal arteries and major splanchnic vessels. [1][2][3][4][5][6] Diagnosis of MAS in premature infants is rare; this report describes the presentation and clinical course in two such patients.…”