Peri-procedural risk stratification and management of patients with Williams syndrome

R. Thomas Collins II MD, Margaret G. Collins DDS, Michael L. Schmitz MD, Justin T. Hamrick

Abstract: Williams syndrome (WS) is a congenital, multisystem disorder affecting the cardiovascular, connective tissue, and central nervous systems in 1 in 10 000 live births. Cardiovascular involvement is the most common cause of morbidity and mortality in patients with WS, and noninvasive and invasive procedures are common. Sudden cardiovascular collapse in patients with WS is a well-known phenomenon, especially in the peri-procedural period.