Research: Use of treprostinil in pediatric pulmonary hypertension: case reports and review of the literature.

J Cardiovasc Pharmacol. 2020 Mar 9. doi: 10.1097/FJC.0000000000000820. [Epub ahead of print]

Use of treprostinil in pediatric pulmonary hypertension: case reports and review of the literature.

Gavotto A1,2Thomas F1Werner O1Moreau J1,2Amedro P1,2.

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Add-on therapy with prostacyclin in pediatric refractory pulmonary hypertension poses a challenge, especially when considering continuous intravenous administration in younger children. A search for alternate routes of drug delivery has led to the clinical investigation of stable and long-acting prostacyclin analogues, such as subcutaneous treprostinil. We reported two pediatric cases of pulmonary hypertension treated with subcutaneous treprostinil and reviewed the literature on treprostinil use in children.


The literature review used three electronic databases and a combination of terms (treprostinil, pediatric, pulmonary hypertension, prostanoid, etc.). We also searched for pediatric clinical trials on treprostinil registered on international clinical trial registries.


The reported cases highlighted the multifactorial nature of pulmonary hypertension in pediatrics: a female child with a giant omphalocele, and intra and extracardiac shunts; and a male premature child with a congenital diaphragmatic hernia and long-term pulmonary hypertension. The literature review identified 19 studies reporting treprostinil use in 421 children with various types of pulmonary hypertension (groups 1 and 3). Subcutaneous treprostinil was the most administered formulation, at a mean dose of 40 ng/kg/min. Overall, 12 clinical trials on treprostinil for children with pulmonary hypertension were registered on the clinical trial registries. Most authors concluded that subcutaneous treprostinil was effective, well tolerated, and represented an alternative to intravenous epoprostenol.


Subcutaneous treprostinil may be a useful adjunct in the therapeutic algorithm for children with severe pulmonary hypertension, refractory to oral drugs, and after a complete check-up for all pulmonary hypertension etiologies.PMID: 32168152 DOI: 10.1097/FJC.0000000000000820

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