title: Mid-aortic syndrome: long-term outcome of 36 children.
abstract: 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.
authors: Department of Paediatric Nephrology, University of Bari, Italy. albinatummolo@yahoo.it
authors: Albina
authors: A
authors: Tummolo
authors: Stephen D
authors: SD
authors: Marks
authors: Marike
authors: M
authors: Stadermann
authors: Derek J
authors: DJ
authors: Roebuck
authors: Clare A
authors: CA
authors: McLaren
authors: George
authors: G
authors: Hamilton
authors: Michael J
authors: MJ
authors: Dillon
authors: Kjell
authors: K
authors: Tullus
mesh_terms: D000792:Angiography; D000800:Angioplasty, Balloon; D001011:Aorta / Q000601:surgery*; D001012:Aorta, Abdominal / Q000000981:diagnostic imaging* / Q000601:surgery*; D001018:Aortic Diseases / Q000473:pathology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D013577:Syndrome; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures