Middle aortic syndrome—an 8-year story of pills, pretty balloons and struts

Elisabeth Day, Jelena Stojanovic, Narayan Karunanthy, Shakeel Qureshi, John Reidy, Manish D. Sinha

Middle aortic syndrome (MAS) is an uncommon cause of hypertension in children. The management of hypertension secondary to MAS frequently requires several anti-hypertensive medications along with endovascular and often surgical intervention. A 9-year-old boy presented with headaches and vomiting and was diagnosed with severe hypertension secondary to idiopathic MAS affecting a long segment of the abdominal aorta and left renal artery stenosis. Over the following 8 years his hypertension was successfully managed initially with percutaneous transluminal balloon angioplasty (PTA) of his left renal artery, followed by balloon dilatation of his abdominal aortic narrowing. He subsequently underwent abdominal aortic stent placement following failed repeat aortic balloon dilatation. Aged 17 years and 6 months he is now on a single anti-hypertensive agent with normal renal function and no evidence of target organ damage. In selected cases, MAS may be managed with PTA and stent placement with good long-term outcomes. Limited data on the use of PTA and stent insertion in children means that a structured approach to the management of refractory hypertension secondary to MAS remains elusive.