Middle Aortic Syndrome: Distal Thoracic and Abdominal Coarctation, a Disorder with Multiple Etiologies1

Connolly, John E.; Wilson, Samuel E.; Lawrence, Peter; Fujitani, Roy M.

doi:10.1016/s1072-7515(02)01144-4

Cited by 162 publications

(150 citation statements)

References 9 publications

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“…In such patients, other mechanisms are to be considered. Middle aortic syndrome causing acquired atypical coarctation and hypertension have been described (22). Arterial baroreceptor abnormalities can be hypothesized considering the high frequency of carotid arteries and aortic arch involvement.…”

mentioning

confidence: 99%

Takayasu's arteritis: A study of 104 Italian patients

Vanoli

Daina

Salvarani³

et al. 2005

Arthritis & Rheumatism

276

158

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Objective. Takayasu's arteritis (TA) is a rare vasculitis. The Italian Takayasu's Arteritis study group was established with the aim to describe a large cohort of patients. Methods. Data were collected by means of an ad hoc form. Demographic information, clinical history, vascular findings, treatment, risk factors, and comorbidities were analyzed. Results. Data of 104 patients were collected. The median delay in diagnosis was 15.5 months (range 0 -325 months). Age at onset <15 years was associated with a higher probability, whereas elevated erythrocyte sedimentation rate with a lower probability, of a delay in diagnosis. The majority of patients experienced nonspecific signs and symptoms indicative of an inflammatory disease in the early phase. Among vascular involvement, stenosis was the most frequent lesion, being present in 93% of patients, followed by occlusion (57%), dilatation (16%), and aneurysm (7%). Glucocorticoids were the mainstay of treatment in our series; however, treatment with cytotoxic agents was required in about half of the patients. Fifty-two patients underwent at least 1 surgical procedure. The main indications for intervention were renal vascular hypertension, cerebral hypoperfusion, and limb claudication. Conclusion. As with many rare diseases, delay in diagnosis is an important issue for patients with TA. The increasing occurrence of vascular lesions along with the disease progression put to question the long-term effectiveness of contemporary treatment. These data may be helpful in increasing physicians' awareness to prevent diagnosis delay, update guidelines, and plan future research projects.

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mentioning

confidence: 99%

Takayasu's arteritis: A study of 104 Italian patients

Vanoli

Daina

Salvarani³

et al. 2005

Arthritis & Rheumatism

276

158

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“…It constitutes about 0.5-2% of all the cases of aortic coarctation [3] . It can be congenital or acquaired.…”

Section: Discussionmentioning

confidence: 99%

Middle Aortic Syndrome (MAS) –A Rare Cause Of Hypertension In A Child with Rheumatic Heart Disease

Ali¹

2017

jmscr

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“…Typical symptoms are hypertension in young and lower limb and/or visceral ischemia, which mostly occur later in life [3]. It is a rare condition that accounts for 0.5-2% of all aortic narrowing, most of which occur at the level of the proximal thoracic aorta [4]. Hypertension is the cardinal clinical feature in MAS and is present in more than 90% of the cases.…”

Section: Discussionmentioning

confidence: 99%

Mid-aortic dysplastic syndrome as a rare cause of hypertension in young

Saha¹,

Saha²,

Ranjit³

et al. 2013

IJCRI

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Introduction: Mid-aortic syndrome (MAs), coarctation of abdominal aorta is a rare disease with only 200 reported cases. It is characterized by constriction of distal thoracic and/or abdominal aorta and its branches, therefore is also known as abdominal aortic coarctation. the MAs is characterized radiologically by severe narrowing of abdominal aorta and its branches and most of these patients usually die due to progressive severe hypertension before the age of 35-40 if left untreated. case report: A 13-year-old boy was admitted with persistent headache and vomiting for one month and repeated generalized tonic clonic seizures for two days. His past history was unremarkable. the highlight of the clinical examination blood pressure was 240/150 mmHg in both the upper limbs, and all peripheral pulses were palpable. blood pressure was similar in upper and lower limbs and a systolic bruit heard over the epigastrium. Ultrasonography showed a localized narrowing of a suprarenal segment

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Middle Aortic Syndrome: Distal Thoracic and Abdominal Coarctation, a Disorder with Multiple Etiologies1

Cited by 162 publications

References 9 publications

Takayasu's arteritis: A study of 104 Italian patients

Takayasu's arteritis: A study of 104 Italian patients

Middle Aortic Syndrome (MAS) –A Rare Cause Of Hypertension In A Child with Rheumatic Heart Disease

Mid-aortic dysplastic syndrome as a rare cause of hypertension in young

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