Surgical Treatment of Middle Aortic Syndrome with Takayasu Arteritis or Midaortic Dysplastic Syndrome

Kim, S. M.; Jung, In Mok; Han, Ahram; Min, Sang Il; Lee, Taeseung; Ha, Jong‐Won; Kim, S. J.; Min, Seung-Kee

doi:10.1016/j.ejvs.2015.04.032

Cited by 38 publications

(41 citation statements)

References 25 publications

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“…Hypertension is the major clinical feature of MAS and was present in 94% of the cases reviewed . Symptomatic patients with hypertension may present with mental status change, headache, nosebleed, sleep disturbance, and fatigue . Patients with MAS are often first detected due to refractory hypertension.…”

Section: Discussionmentioning

confidence: 99%

Doppler ultrasonographic and clinical features of middle aortic syndrome

Yan

et al. 2018

J of Clinical Ultrasound

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Objectives To discuss Doppler ultrasonographic and clinical features of middle aortic syndrome (MAS). Materials and methods Doppler ultrasonographic images and clinical dates of 11 patients with MAS confirmed by angiography were retrospectively analyzed from January 2004 to September 2016. Results The median age of 11 patients was 10 years (1‐39 years). Ten patients presented with hypertension, only 2 cases presented with symptomatic intermittent claudication, and 1 case presented with abdominal pain. The ultrasonographic features of 11 patients with MAS included: (a) Gray‐scale image showed significant segmental narrowing of the aorta in 9 cases. (b) Color Doppler demonstrated aliasing in the suspicious narrowed vessels of all cases. (c) On Spectral Doppler image, peak systolic velocity in the location of aorta coarctation was significantly elevated (range, 2.3~4.8 m/s). When infrarenal aorta was involved, a tardus‐parvus waveform was only seen in the distal aorta. When suprarenal or inter‐renal aorta was involved, a tardus‐parvus pattern was seen in the distal aorta as well as renal artery. Conclusions Significant segmental narrowing and a tardus‐parvus waveform are the important ultrasonographic features in patients with MAS, the latter may be more reliable. Doppler ultrasound can be used as a simple screening method, especially for children and adolescents suspected of having a vascular cause of refractory hypertension.

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Section: Discussionmentioning

confidence: 99%

Doppler ultrasonographic and clinical features of middle aortic syndrome

Yan

et al. 2018

J of Clinical Ultrasound

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“…Five years after surgery, all patients remained normotensive and free of symptoms. Kim et al [9] reported that 10 adult patients diagnosed with MAS caused by Takayasuarteritis who underwent surgical treatment. Indications for operation were uncontrolled hypertension in six patients and lower extremity claudication in four.…”

Section: Discussionmentioning

confidence: 99%

Middle aortic syndrome with renal artery stenosis

Chitrakar¹,

Shrestha²,

Shrestha³

2017

Journal of Surgical Case Reports

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A 39-year lady referred to Vascular Surgery OPD from Cardiology with uncontrolled hypertension, non- functional left kidney and absent bilateral femoral pulses. Investigations revealed middle aortic syndrome with non-functional left kidney with bilateral renal artery stenosis. She successfully underwent bypass of stenotic segment with polyester graft from descending thoracic aorta to left common iliac artery with reverse saphenous vein graft interposed between polyester and right renal artery.

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“…Other authors have also used the terms midaortic syndrome or midaortic dysplastic syndrome to describe such lesions 4 5. Ninety-eight per cent of all aortic blockages are at the level of ductus arteriosus, MAS is different from the usual aortic coarctations since it usually affects more distal aortic segments and can also potentially affect long segments, up to several centimetres at a stretch 6.…”

Section: Discussionmentioning

confidence: 99%

“…The acquired form of the disease, on the other hand can potentially present later in life. Kim et al 4 described five patients in the sixth decade of their lives, with MAS secondary to Takayasu's arteritis.…”

Section: Discussionmentioning

confidence: 99%

Unlikely culprit: congenital middle aortic syndrome diagnosed in the sixth decade of life

et al. 2016

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A 58-year-old woman was admitted with acute heart failure. She had a long history of resistant hypertension, with an unremarkable work up for secondary causes in the past. Her brachial blood pressure was 210/70 mm Hg, with ankle blood pressure of 100/70 mm Hg. CT angiogram revealed marked narrowing of the descending thoracic aorta between the left subclavian artery and the diaphragm, consistent with middle aortic syndrome (MAS). She was initially managed with diuretics and antihypertensives. Subsequently thoracotomy revealed a severely hypoplastic segment of the descending aorta. The diseased segment was resected and aortic reconstruction performed. Histopathology showed fragmentation of the medial elastic fibres and fibrosis of the medial and intimal layers. These findings along with gross aortic hypoplasia and absence of features of Takayasu's arteritis, suggest that our patient had congenital MAS. The patient has done well since her surgery. We believe this is the first case of congenital MAS reported in the sixth decade of life.

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Surgical Treatment of Middle Aortic Syndrome with Takayasu Arteritis or Midaortic Dysplastic Syndrome

Abstract: Aortic bypass for MAS is safe and shows excellent long-term durability. Considering the patients are relatively young with a long life expectancy, aggressive surgical treatment could be beneficial. Lifelong follow up to monitor complications and disease progression is necessary.

Cited by 38 publications

References 25 publications

Doppler ultrasonographic and clinical features of middle aortic syndrome

Doppler ultrasonographic and clinical features of middle aortic syndrome

Middle aortic syndrome with renal artery stenosis

Unlikely culprit: congenital middle aortic syndrome diagnosed in the sixth decade of life

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