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.
Keywords: implantable pump; pediatrics; prostanoid treatment; pulmonary arterial hypertension.
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