Abstract
Pulmonary hypertension contributes significantly to the morbidity and mortality of diverse cardiopulmonary and systemic disorders in children. Advances in vascular biology have led to the identification and characterization of potent endothelium-derived vasoactive mediators, which include nitric oxide (NO), prostacyclin (PgI2) and endothelin-1 (ET-1), that play critical roles in the regulation of pulmonary vascular tone, reactivity, growth and structure. Insights into these agents and their signaling pathways have led directly to the development of current therapies for severe pulmonary hypertension. In this chapter, I will briefly review basic pharmacologic choices for the general management of chronic pulmonary hypertension in older children (outside of the neonatal period). Guidelines and discussion of relevant literature has been recently reviewed.
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