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

Albina Tummolo1, Stephen D Marks, Marike Stadermann

  • 1Department of Paediatric Nephrology, University of Bari, Italy. albinatummolo@yahoo.it

Pediatric Nephrology (Berlin, Germany)
|July 16, 2009
PubMed

Insights

Mid-aortic syndrome (MAS) in children is a severe condition affecting multiple arteries. Effective treatment combining medical, angioplasty, and surgical interventions significantly reduces blood pressure and preserves kidney function in most survivors.

Area of Science:

  • Pediatric Nephrology
  • Pediatric Cardiology
  • Vascular Surgery

Background:

  • Mid-aortic syndrome (MAS) is a rare congenital condition characterized by narrowing of the abdominal aorta, often associated with other vascular and systemic abnormalities.
  • Clinical presentation and long-term outcomes of pediatric MAS, particularly regarding hypertension management and renal function, require further elucidation.

Purpose of the Study:

  • To review the clinical characteristics and outcomes of children diagnosed with MAS.
  • To evaluate the effectiveness of various therapeutic strategies, including medical management, percutaneous transluminal angioplasty, and surgical intervention, in managing hypertension and preserving renal function in pediatric MAS patients.

Main Methods:

  • A single-center retrospective review of medical records for children with MAS diagnosed via angiography over a 30-year period.
  • Data collection included patient demographics, associated syndromes, cerebrovascular disease, arterial involvement, therapeutic interventions, mortality, blood pressure control, and estimated glomerular filtration rate (eGFR).

Main Results:

  • Thirty-six children with MAS were analyzed, presenting at a median age of 2.7 years. Associated syndromes were present in 36% and cerebrovascular disease in 44%. All patients had multi-arterial involvement.
  • The overall mortality rate was 8%. Among survivors, 90% achieved blood pressure reduction. At last follow-up, 76% had normal eGFR, while 17% presented with renal dysfunction.
  • Effective blood pressure control and preservation of renal function were observed following a combination of medical, angioplasty, and surgical interventions.

Conclusions:

  • Mid-aortic syndrome is a severe, widespread pediatric vascular disease with significant morbidity.
  • A multimodal therapeutic approach combining medical management, angioplasty, and surgery offers effective control of hypertension and preserves renal function in the majority of pediatric MAS patients.
  • Despite its severity, MAS can be managed effectively, leading to improved long-term outcomes for surviving children.

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