Clinical Care for Cardiovascular Disease in Patients With Williams–Beuren Syndrome

Figure 1. Supravalvar aortic stenosis in Williams–Beuren syndrome. A and C, Discrete form of supravalvar aortic stenosis. B and D, Long‐segment supravalvar aortic stenosis that extends to the descending thoracic aorta.

Figure 2. Pulmonary artery stenosis in Williams–Beuren syndrome. A, Diffuse stenosis of the branch pulmonary arteries and all subsequent branches to the subsegmental level. B, Focal stenoses at the branching points of the pulmonary arteries.

Figure 3. Coronary artery stenosis in Williams–Beuren syndrome. A, Severe stenosis of the proximal left main coronary artery. The origin of the coronary artery is at the level of the sinotubular junction, where there is supravalvar aortic stenosis. The distal segments of the left anterior descending and circumflex coronary arteries are diffusely narrowed. B, Severe stenosis of the left main coronary artery with an origin near the sinotubular junction.

Figure 4. Renal artery stenosis in Williams–Beuren syndrome. A, A 3‐dimensional rendered computed tomographic angiogram of the descending abdominal aorta and renal arteries. There is severe stenosis of the origins of the right and left renal arteries with larger calibers more distally. B, Abdominal aortic angiogram. There is severe diffuse stenosis of bilateral renal arteries.

Figure 5. Long‐segment stenosis of the thoracic aorta in Williams–Beuren syndrome. A, A 3‐dimensional rendered computed tomographic angiogram of severe stenosis of the aortic arch and proximal descending thoracic aorta. B, Angiogram of the thoracic aorta with stenosis from the ascending aorta to the diaphragm.