Dynamic Left Main Coronary Artery Obstruction Associated With Williams Syndrome

Abstract

A 5-year-old boy with Williams syndrome (WS) presented with a history of diffuse recurrent obstructive vascular disease. At the age of 12 months, he underwent pericardial patch augmentation of the ascending aorta and pulmonary arteries. He subsequently required Dacron patch repair of the ascending aorta and aortic arch at 3 years of age. In the interim, he had undergone percutaneous transluminal angioplasty and stenting for recurrent peripheral pulmonary artery stenoses and aortic recoarctation. On routine follow-up, he was found to have worsening right ventricular hypertension associated with disparate lung perfusion on a nuclear perfusion scan. He was brought to the catheterization laboratory for further investigation. Angiography showed recurrent pulmonary artery in-stent restenosis and aortic recoarctation. Coronary …