Key result
Middle aortic syndrome is a rare form of renovascular disease causing severe childhood hypertension that often requires endovascular or surgical intervention when medical therapy is insufficient.
Middle aortic syndrome in children requires a multidisciplinary approach involving medical, endovascular, and surgical therapies to manage severe hypertension and prevent end-organ damage.
Middle aortic syndrome is a rare form of renovascular disease that may present with severe hypertension during childhood. Narrowing of the abdominal aorta is often associated with narrowing of the renal and/or other visceral arteries and may be secondary to specific genetic syndromes. Following the optimization of blood pressure control, significant aortic narrowing often requires invasive management, including endovascular and surgical intervention. In younger children, endovascular therapy may be attempted in the first instance to acutely reduce the pressure gradient across the narrowing; however, a sustained benefit is rare. Once the child has grown to accommodate a graft of an adequate size, surgical therapy is indicated for patients in whom medical and/or endovascular management has not resulted in adequate blood pressure control. It is critical that individuals with middle aortic syndrome be managed by an experienced multidisciplinary team that includes medical, endovascular, and surgical expertise that can provide long-term care to monitor for recurrent hypertension and evidence of end-organ damage.
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Musajee et al. (2022) conducted a review in Middle aortic syndrome. Medical, endovascular, and surgical management was evaluated. Middle aortic syndrome is a rare form of renovascular disease causing severe childhood hypertension that often requires endovascular or surgical intervention when medical therapy is insufficient.
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