Middle aortic syndrome: clinical and radiological findings

Eight patients with the middle aortic syndrome are described. They were aged 2 months to 14 years at diagnosis; follow up was one to 11 years. Clinical presentations included asymptomatic hypertension (n = 5), severe headache, nose bleed, and chest pain (n = 1), and cardiac failure (n = 1). All had...

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Bibliographic Details
Published in:Archives of disease in childhood 1992-04, Vol.67 (4), p.501-505
Main Authors: Sumboonnanonda, A, Robinson, B L, Gedroyc, W M, Saxton, H M, Reidy, J F, Haycock, G B
Format: Article
Language:English
Subjects:
Adolescent
Antihypertensive Agents - therapeutic use
Aorta, Abdominal - diagnostic imaging
Aortic Diseases - complications
Aortic Diseases - diagnostic imaging
Aortic Diseases - drug therapy
Biological and medical sciences
Blood and lymphatic vessels
Cardiology. Vascular system
Child
Child, Preschool
Diseases of the aorta
Female
Humans
Hypertension - drug therapy
Hypertension - etiology
Infant
Male
Medical sciences
Radiography
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Summary:Eight patients with the middle aortic syndrome are described. They were aged 2 months to 14 years at diagnosis; follow up was one to 11 years. Clinical presentations included asymptomatic hypertension (n = 5), severe headache, nose bleed, and chest pain (n = 1), and cardiac failure (n = 1). All had severe hypertension requiring multiple drug treatment. Diminished peripheral pulses were not helpful in the diagnosis, which is made on aortography. Associated clinical findings were Williams' syndrome (n = 3) and appreciable eosinophilia (n = 3). The differential diagnosis includes Takayasu's arteritis, fibromuscular dysplasia, and neurofibromatosis. Blood pressure was adequately controlled by medical treatment in six patients. Surgical angioplasty was performed in two. One patient remained normotensive without drug treatment 21 months after operation; the other died of sepsis and uncontrollable haemorrhage in the postoperative period. Medical treatment is satisfactory in most cases: surgery should be reserved for those in whom blood pressure cannot be controlled without unacceptable side effects of drug treatment. Although rare, the middle aortic syndrome should be considered in the differential diagnosis of hypertension when commoner causes have been excluded. Aortography is necessary for diagnosis.
ISSN:0003-9888
1468-2044
DOI:10.1136/adc.67.4.501