Non-congenital heart disease associated pediatric pulmonary arterial
hypertension.
Author(s): Ivy DD(1), Feinstein JA, Humpl T, Rosenzweig EB.
Affiliation(s): Author information:
(1)University of Colorado Denver School of Medicine and The Children's Hospital,
United States.
Publication date & source: 2009, Prog Pediatr Cardiol. , 27(1-2):13-23
Recognition of causes of pulmonary hypertension other than congenital heart
disease is increasing in children. Diagnosis and treatment of any underlying
cause of pulmonary hypertension is crucial for optimal management of pulmonary
hypertension. This article discusses the available knowledge regarding several
disorders associated with pulmonary hypertension in children: idiopathic
pulmonary arterial hypertension (IPAH), pulmonary capillary hemangiomatosis,
pulmonary veno-occlusive disease, hemoglobinopathies, hepatopulmonary syndrome,
portopulmonary hypertension and HIV. Three classes of drugs have been extensively
studied for the treatment of IPAH in adults: prostanoids (epoprostenol,
treprostinil, iloprost, beraprost), endothelin receptor antagonists (bosentan,
sitaxsentan, ambrisentan), and phosphodiesterase inhibitors (Sildenafil,
tadalafil). These medications have been used in treatment of children with
pulmonary arterial hypertension, although randomized clinical trial data is
lacking. As pulmonary vasodilator therapy in certain diseases may be associated
with adverse outcomes, further study of these medications is needed before
widespread use is encouraged.
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