Implantable LENUS pro pump for treprostinil infusion in three pediatric patients

Abstract

Background Prostanoid treatment in patients with severe pulmonary arterial hypertension (PAH) has been proven safe and effective. Subcutaneous administration of treprostinil has side effects, which limits their use and acceptance. An implantable pump for continuous intravenous treprostinil infusion has been recently approved. We describe our experience with the implantable pump in three pediatric patients. Description of Cases The LENUS pro pump was implanted in three adolescents with severe PAH, who were treated with tadalafil, ambrisentan, and subcutaneous treprostinil. The indication of the Lenus pro pump implantation was the local side effects of subcutaneous treprostinil (pain, inflammation, and local infection) that were not well tolerated and that severely decreased their quality of life. The pump was surgically implanted under general anesthesia.One patient, in functional class IV, suffered postoperative hemodynamic instability and small pneumothorax, requiring an increase in treprostinil dose up to 85 ng/kg/min and a decrease 9 days after the pump implantation. The second patient who was discharged 4 days after surgery with treprostinil at 60 ng/kg/min reported improvement in his quality of life, but the dose requirement increased up to 92 ng/kg/min. After a 21‐month follow‐up, this patient received a lung transplant. The third patient presented a hematoma at the pump site with no other complications and had a follow‐up of 9 months with an improvement in her quality of life. Comments Implantable pumps for continuous parenteral prostanoid infusion in pediatric patients are an alternative to external pumps, especially when familiar psychological or psychomotor issues hinder the use of external pumps.

Off‐label use of PAH‐targeted medications approved for adults and their financial coverage by health insurances are vital for children with pulmonary hypertension

“…In addition, off-label subcutaneous implantation of an intravenous treprostinil pump for continuous drug delivery has been reported in very few adolescents (Lenus Pro: 20mL, 40mL). 57,58 Even after successful implementation of one of these invasive measures, only bilateral lung transplantation (LuTx) remains a therapeutic option in the far advanced stages of PAH and associated RV failure. However, allograft rejection is common and organ half-life is rather short after LuTx compared to other organ transplantations.…”

“…In addition to pharmacotherapy, 24,29,34,47 a catheter interventional or surgical procedure, such as the establishment of a reverse Potts shunt 55,56 or an atrial septostomy, may also be considered for selected PAH patients, 29 although they carry a considerable risk of procedure‐related death or morbidity. In addition, off‐label subcutaneous implantation of an intravenous treprostinil pump for continuous drug delivery has been reported in very few adolescents (Lenus Pro: 20mL, 40mL) 57,58 . Even after successful implementation of one of these invasive measures, only bilateral lung transplantation (LuTx) remains a therapeutic option in the far advanced stages of PAH and associated RV failure.…”

Opportunities and challenges of pharmacotherapy for pulmonary arterial hypertension in children

“…However, commercial launch in adults has been pushed back due to the FDA's request to complete a postapproval study 56,57 (NCT03833323). Successful administration of IV treprostinil via other implantable pumps, like the Lenus Pro®, in European pediatric patients has been described 58,59 . Additionally, in May 2019, the FDA approved the Remunity SQ pump (DEKA Research and Development Corp and United Therapeutics) for patients 22 years and older, with plans for commercial release later in 2020.…”