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.

Renal artery stenosis: is angiography still the gold standard in 2011?

“…In a quarter of our children the main problem was midaortic syndrome (MAS) [5]. Twenty percent of children have major involvement both extra-and intra-cerebral blood vessels.…”

“…The imaging should be done in a stepwise fashion. It should consider the need to investigate several vascular beds as a majority of children with RAS display involvement of multiple arteries [3][4][5]. At least 50% of the children have bilateral RAS; some have mainly involvement at the arterial ostium, while others have RAS in smaller intrarenal arteries, both of which can be difficult to diagnose on non-invasive imaging.…”

“…A further reason for the lack of major improvement in the blood pressure despite technically good results is that a large proportion of children that are treated have involve- ment of several other blood vessels including the intra-renal arteries and the aorta (mid-aortic syndrome) [5,13].…”

Mid-aortic syndrome in two preterm infants

“…7,8 Patients with MAS are usually diagnosed during childhood or adolescence though presentation in infancy has been reported. [9][10][11][12][13][14][15][16] It is speculated that this recent trend towards earlier age at presentation may be the result of improved diagnostic methods and an increase in premature births. 10 Preterm infants diagnosed with MAS likely have a very poor prognosis.…”

“…[9][10][11][12][13][14][15][16] It is speculated that this recent trend towards earlier age at presentation may be the result of improved diagnostic methods and an increase in premature births. 10 Preterm infants diagnosed with MAS likely have a very poor prognosis. Both previously reported preterm infants with MAS died as a result of developing sequelae of severe hypertension, one due to cardiac dysfunction, the other as a consequence of IVH.…”