Pulmonary Stenosis

Pulmonary stenosis, defined as an obstruction to outflow of blood from the heart to the pulmonary vasculature, is an heterogenous entity. Obstruction can be at the valvar, subvalvar, or supravalvar level. While pulmonary stenosis may be isolated, it can also be a component of other congenital heart diseases, or associated with numerous genetic syndromes. Disease severity ranges from the neonatal form of ductal-dependent critical pulmonary stenosis to a milder form that is well-tolerated for prolonged periods. For the majority of patients with isolated valvar pulmonary stenosis, catheter-based therapy is curative. Patients with subvalvar and supravalvar obstruction commonly require primary surgical therapy with ongoing catheter-based surveillance and interventions. In this chapter, we discuss these defects along with some common principles that determine current management and outcomes.