Subcutaneous Treprostinil for Pulmonary Hypertension in Chronic Lung Disease of Infancy

Abstract

Pulmonary arterial hypertension (PAH) associated with chronic lung disease of infancy can be a life-threatening disease affecting an increasing number of former premature infants. There is a need for improved delivery of targeted PAH therapies for this subgroup of patients who have severe and persistent PAH despite standard respiratory care for chronic lung disease. Currently infants who have severe PAH despite oral or inhaled therapy receive continuous intravenous prostanoid therapy (mostly epoprostenol), which is complicated because of the need for central venous access and associated catheter-related complications. We present a series of 5 infants who were successfully treated with a continuous infusion of subcutaneous treprostinil, which is a longeracting prostanoid with similar hemodynamic effects. There were improvements in echocardiographic assessment of right ventricular function and estimated pulmonary hypertension, and in respiratory support required within weeks of therapy. Unlike commonly in adults, these 5 infants had no instances of severe site erythema, bleeding, bruising, or infection. In our experience with 5 former extremely preterm infants who had PAH associated with chronic lung disease, subcutaneous treprostinil was safe, efficacious, and well tolerated. We believe that subcutaneous treprostinil can be beneficial in a select group of former premature infants who have chronic lung disease and severe pulmonary arterial hypertension who have not responded adequately to conservative therapies. Pediatrics 2014;134:e274-e278 AUTHORS:

Advances in Neonatal Pulmonary Hypertension

“…New, more stable preparations are emerging that are specifically designed for intermittent nebulization, such as iloprost or treprostinil. Treprostinil is particularly promising because it is also suitable for systemic administration, including by the subcutaneous route [62]. A randomized study of intravenous remodulin for PPHN and CDH recently began enrolling patients (NCT02261883).…”

Idiopathic Pulmonary Arterial Hypertension in Children: A Review

“…Subcutaneous treprostinil has equivalent pharmacokinetics to intravenous and is an attractive option because of ease of administration not needing an indwelling intravenous catheter. One of the most common adverse effects is discomfort at the administration site, which is less frequent in children compared to adults [127,128].…”

Pulmonary Hypertension in Children

“…114 Subcutaneous treprostinil in young children is well tolerated in many children with tolerable side effects. 115,116 Treprostinil has also been studied in an inhaled form. 117–119 Oral treprostinil was shown to effective as initial monotherapy treatment in adult PAH 120 , but not as add-on therapy.…”