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Mid-aortic syndrome: long-term outcome of 36 children.

Tummolo, A; Marks, SD; Stadermann, M; Roebuck, DJ; McLaren, CA; Hamilton, G; Dillon, MJ; (2009) Mid-aortic syndrome: long-term outcome of 36 children. Pediatr Nephrol , 24 (11) pp. 2225-2232. 10.1007/s00467-009-1242-6.

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The clinical characteristics and outcomes of children with mid-aortic syndrome (MAS) and the effectiveness of different therapeutic approaches in reducing hypertension are still debated. We conducted a single-centre retrospective review of the records of children with MAS over 30 years. Children with angiographic evidence of a narrowed abdominal aorta were included. Therapeutic approaches included medical management, percutaneous transluminal angioplasty and/or surgical intervention. Thirty-six children had presented at a median age of 2.7 years (10 days-10 years). Thirteen (36%) patients had associated syndromes, and 44% had been diagnosed with cerebrovascular disease. All patients had involvement of multiple arteries. The mortality rate was 8% after a median follow-up period of 4.5 (range 1.1-19.7) years. Among the children who survived, 90% had obtained a reduction in their blood pressure (BP). Of the patients, 76% had had a normal estimated glomerular filtration rate (eGFR) at the last follow-up examination. Seventeen percent (six of 36) had renal dysfunction at presentation. Although MAS is a severe and widespread disease, in most cases it can be effectively treated with a combination of medical, angioplasty and surgical interventions.

Type: Article
Title: Mid-aortic syndrome: long-term outcome of 36 children.
Location: Germany
DOI: 10.1007/s00467-009-1242-6
Keywords: Angiography, Angioplasty, Balloon, Aorta, Aorta, Abdominal, Aortic Diseases, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Male, Retrospective Studies, Syndrome, Time Factors, Treatment Outcome, Vascular Surgical Procedures
URI: http://discovery.ucl.ac.uk/id/eprint/89853
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