R. Thomas Collins MD, May 2013
WS is a complex, multisystem disorder with significant cardiovascular manifestations. Arterial stenoses make up the large majority of cardiovascular issues in patients with WS. Although all patients with WS need early and ongoing cardiovascular evaluation and follow-up, most patients will not require cardiovascular interventions. In those patients who do undergo surgical and catheter-based interventions, the results are favorable, although long-segment STA remains very difficult to treat.
Sudden death in patients with WS is significantly greater than in the general population, and expert periprocedural care of these patients is paramount. Prolongation of the QTc is common in patients with WS, and conservative management with medications should be considered. The potential for groundbreaking advancements in medical therapies is present in already available pharmaceutical agents; these potential therapies need to be investigated in meaningful ways.