Dysphagia: An unusual presentation of giant aneurysm of the right coronary artery and supravalvular aortic stenosis in Williams syndrome

Clinical Summary A 56-year-old patient with dysphagia was admitted to our hospital for evaluation. On medical history, he was found to have neurodevelopmental delay and was receiving antihypertensive therapy. An extrinsic stenosis on the midportion of the esophagus did not allow us to complete full gastroenteral screening. Computed tomographic scanning showed the presence of a mediastinal mass 11 cm in diameter compressing the esophagus (Figure 1), which was diagnosed as an isolated aneurysm of the right sinus of Valsalva. The echocardiogram showed the presence of an SVAS with a gradient of 70 mm Hg, and the pulmonary trunk and bifurcation were normal. Angiography showed normal peripheral pulmonary vascularization and normal left coronary artery anatomy. The right coronary artery at its origin from the aorta opened up in a huge dilatation of 11 cm. The patient was taken to the operating theater on an urgent basis. Cardiopulmonary bypass was instituted through femorofemoral cannulation. The heart was emptied, and the chest was opened. At opening, it was possible to visualize a huge aneurysmal mass compressing the right atrium and superior vena cava and covering the ascending aorta (Figure 2, A). The aorta was crossclamped and transected. Warm blood cardioplegic solution was administered directly into the coronary ostia. When the aorta