Pediatric Pulmonary Hypertension: A Pharmacotherapeutic Review

Abstract

Pulmonary hypertension in children is a disorder associated with increased pulmonary vascular resistance and arterial pressure, decreased cardiac output, and right-sided cardiac dysfunction that is caused by numerous etiologies. Although treatment will vary with underlying cause, pharmacological treatment has historically included inhaled nitric oxide and prostacyclin analogues. Over the past several years new agents have been added to the treatment armamentarium, including phosphodiesterase V inhibitors (eg sildenafil) and endothelin antagonists (eg bosentan). Further, more agents are currently under investigation for pulmonary hypertension in children including immunosuppressives and other endothelin antagonist entities. Limitations to treatment include the availability of appropriate, robust pediatric pharmacological data and constraints with dosage forms.