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Infantile midaortic syndrome with aortic occlusion
Susan Minson1, Clare A McLaren, Derek J Roebuck
1Department of Paediatric Nephrology, Great Ormond Street Hospital for Children, Great Ormond Street, London, UK, WC1N 3JH.
Mid aortic syndrome (MAS), a rare condition causing abdominal aorta narrowing, can be successfully treated in infants. A combination of medical management and angioplasty improved a severe case, leading to normal blood pressure and quality of life.
Area of Science:
- Pediatric Cardiology
- Vascular Surgery
Background:
- Mid aortic syndrome (MAS) is a rare congenital condition characterized by narrowing of the abdominal aorta, often leading to severe hypertension.
- Early diagnosis and intervention are crucial for managing MAS, particularly in infants presenting with critical symptoms like heart failure.
Observation:
- A case of complete abdominal aortic occlusion presenting in an infant at four months of age with severe hypertension and heart failure.
- Initial assessment deemed the condition too severe for surgical intervention, with palliative care considered.
Findings:
- Successful angioplasty was performed at five and a half months, followed by three additional procedures.
- The patient required antihypertensive agents but maintained normal renal function.
- At six years old, the child exhibits normal growth, development, and excellent quality of life with controlled blood pressure.
Implications:
- This case highlights the efficacy of combining medical management with endovascular procedures like angioplasty for severe infantile MAS.
- Aggressive interventional treatment can lead to favorable long-term outcomes, including normal development and quality of life, even in complex cases.
- It suggests that even seemingly inoperable cases of MAS may benefit from a multidisciplinary approach involving interventional cardiology and medical therapy.
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