Pediatric Idiopathic Pulmonary Arterial Hypertension with Mental Retardation Successfully Treated with the Introduction and Maintenance of a Continuous Subcutaneous Infusion of Treprostinil

Abstract

Treprostinil, a prostacyclin analogue, is administered subcutaneously and intravenously as a treatment for pulmonary arterial hypertension in Japanese adults. To the best of our knowledge, this is the first report to describe the successful subcutaneous infusion of treprostinil in a pediatric Japanese patient. The patient was diagnosed with idiopathic pulmonary arterial hypertension (IPAH) at the age of 7 years. Initial treatments included a combination therapy of three oral drugs and home oxygen therapy. When we determined that the treatment was insufficient, we decided to add subcutaneous treprostinil because administering intravenous epoprostenol would have been difficult due to the patient being mentally retarded. World Health Organization functional classification and echocardiographic findings showed improvement 6 months after the initiation of treprostinil. Therefore, we suggested that subcutaneous treprostinil is effective for children with IPAH and suggest that this drug is used for pediatric patients who cannot tolerate intravenous epoprostenol, or who can withdraw from epoprostenol.