Abstract
Pulmonary Hypertension (PH), the syndrome of elevated pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children. PH is associated with a wide variety of potential underlying causes, including cardiac, pulmonary, hematologic and rheumatologic abnormalities. Regardless of the cause, for many patients the natural history of PH involves progressive elevation in pulmonary arterial resistance and pressure, right ventricular dysfunction, and eventually heart failure. In recent years, a number of pulmonary arterial hypertension (PAH)-targeted therapies have become available to reduce pulmonary artery pressure and improve outcome. A growing body of evidence in both the adult and pediatric literature demonstrates enhanced quality of life, functional status, and survival among treated patients. This review provides a description of select etiologies of PH seen in pediatrics and an update on the most recent data pertaining to evaluation and management of children with PH/PAH. The available evidence for specific classes of PAH-targeted therapies in pediatrics is additionally discussed.
Highlights
Untreated, pulmonary arterial hypertension (PAH) in children carries a poor prognosis
In 1999, a study by Barst et al showed that survival for children with severe idiopathic PAH (IPAH) who were candidates for intravenous prostacyclin but were unable to be treated with this therapy was poor (45% and 29% at 1 and 4 years, respectively) [2]
Unlike what is seen among adult patients, pediatric pulmonary hypertension is intrinsically linked to lung growth and development [6]
Summary
Pulmonary arterial hypertension (PAH) in children carries a poor prognosis. For patients whose disease progresses despite maximal medical therapy, the surgical options include lung transplantation or a Potts shunt—creation of a left pulmonary artery to descending aorta connection in an effort to facilitate right-to-left shunting [9]. This procedure can provide effective palliation in carefully selected children with end-stage pulmonary vascular disease and suprasystemic PAH [10]. Patients compiled at 3 pulmonary hypertension (PH) centers (Denver, New York, Netherlands): 1-, 3-, 5- and 7-year transplantation-free survival rates were 96%, 89%, 81% and 79%, respectively [4]
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