title: Sildenafil in pediatric pulmonary arterial hypertension.
abstract: Pulmonary arterial hypertension (PAH) is a life-threatening disease of varied etiologies. Although PAH has no curative treatment, a greater understanding of pathophysiology, technological advances resulting in early diagnosis, and the availability of several newer drugs have improved the outlook for patients with PAH. Sildenafil is one of the therapeutic agents used extensively in the treatment of PAH in children, as an off-label drug. In 2012, the United States Food and Drug Administration (USFDA) issued a warning regarding the of use high-dose sildenafil in children with PAH. This has led to a peculiar situation where there is a paucity of approved therapies for the management of PAH in children and the use of the most extensively used drug being discouraged by the regulator. This article provides a review of the use of sildenafil in the treatment of PAH in children.
authors.affiliation: Department of Pediatrics, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India.
authors.forename: A K
authors.initials: AK
authors.lastname: Dhariwal
authors.forename: S B
authors.initials: SB
authors.lastname: Bavdekar
mesh_terms: D002648:Child; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D000068677:Sildenafil Citrate / Q000009:adverse effects* / Q000494:pharmacology* / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000009:adverse effects* / Q000494:pharmacology* / Q000627:therapeutic use
chemical_list: D014665:Vasodilator Agents; D000068677:Sildenafil Citrate