The middle aortic syndrome: an important feature of Williams' syndrome

The middle aortic syndrome, with diffuse narrowing of the thoracic and abdo minal aorta, was present in 10 of 18 patients with Williams' syndrome (55%) . There were 3 thoracic coarctations, and 2 abdominal coarctations, with gr adients greater than 20 mmHg across the tone of narrowing. Seven patients h ad mild renal arterial stenosis, and 6 had visceral arterial stenoses. Ten were hypertensive. Measured dimensions of the aortic lumen failed to increa se with age in ? males who had serial angiographic studies. One developed m esenteric arterial stenosis, with mild bilateral renal arterial stenoses, b etween the ages of 9 and 19 years. Aortic intravascular ultrasound performe d in 2 patients confirmed abnormally thick vessel walls with small lumens. Diffusely narrowed and thick-walled stiff arteries, lacking elastin, are a feature of Williams' syndrome. The arteriopathy tends to progress with age, and systemic hypertension is common in teenagers and beyond. The middle ao rtic syndrome was present in more than half our patients, and does not nece ssarily reflect a bias because of cardiologic referral. Aortography with me asurement of aortic diameters and delineation of the visceral branches is a n important requirement for complete evaluation of patients with Williams' syndrome.