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Intravenous treprostinil via an implantable pump in pediatric pulmonary arterial hypertension.
- Source :
-
Pulmonary circulation [Pulm Circ] 2019 Jan-Mar; Vol. 9 (1), pp. 2045894018788846. Date of Electronic Publication: 2018 Jun 26. - Publication Year :
- 2019
-
Abstract
- Intravenous prostacyclin-based therapy improves survival in children with pulmonary arterial hypertension (PAH), but is typically administered via an external infusion pump, which places a considerable burden on the patient. Implanted pumps may overcome some of the limitations of external pumps. We describe the first long-term use of an implanted pump for intravenous treprostinil delivery in a pediatric patient with PAH. Our patient was experiencing marked dyspnea on exertion despite triple combination therapy with bosentan, sildenafil, and inhaled iloprost. Parenteral prostacyclin-based therapy was discussed and the patient rejected options involving external pumps; she finally chose intravenous treprostinil delivery via an implanted pump (LENUS Pro®; fixed flow rate; 20 ml reservoir). The patient underwent pump implantation in July 2012 (aged 14 years) under general anesthesia with no peri- or postoperative complications. She showed marked improvements in fatigue and dyspnea over the subsequent weeks, and her inhaled iloprost regimen was slowly decreased and stopped after six months. During follow-up, the pump showed an unexpected, progressive increase in flow rate that allowed a treprostinil dose of 170 ng/kg/min to be achieved, but at the cost of shortened intervals between refills. The pump was therefore replaced in August 2017 with a newer model with an adjustable flow rate (Siromedes®). A catheter dislocation was corrected under local anesthesia one week after the replacement surgery. The patient is currently receiving treprostinil 170 ng/kg/min with percutaneous refills every 12-13 days. Thus, implantable pumps might be a valuable alternative to external pumps for treprostinil infusion in pediatric PAH.
Details
- Language :
- English
- ISSN :
- 2045-8932
- Volume :
- 9
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Pulmonary circulation
- Publication Type :
- Report
- Accession number :
- 29944075
- Full Text :
- https://doi.org/10.1177/2045894018788846