Pulmonary hypertension in children

Summary 

Pulmonary hypertension in children previously carried a poor prognosis. Recent advances in the understanding of normal and hypertensive pulmonary circulations have led to the introduction of pharmacological therapies and, in turn, this has led to an improved prognosis for children with this condition. The diagnosis and evaluation of Pulmonary hypertension in children is discussed and a regimen for treatment is outlined with a rational for the use of pharmacological agents. The selection of appropriate therapies is complex, requiring familiarity with the underlying disease process, complicated delivery systems and dosing regimens, and medication complications.

Keywords: Idiopathic (primary) pulmonary arterial hypertension (IPAH), Eisenmenger syndrome, Familial pulmonary arterial hypertension, Nitric oxide, Calcium channel blocker, Prostacycline, Endothelin receptor antagonists, Phosphodiesterase-5 inhibitors