Totally Implantable IV Treprostinil Therapy in Pulmonary Hypertension Assessment of the Implantation Procedure

Waxman, Aaron B.; McElderry, H. Thomas; Gomberg‐Maitland, Mardi; Burke, Martin C.; Ross, Edgar L.; Bersohn, Malcolm; Pangarkar, Sanjog; Tarver, James; Zwicke, Dianne; Feldman, Jeremy; Chakinala, Murali M.; Frantz, Robert P.; Thompson, Geoffrey B.; Torres, Fernando; Rauck, Richard; Clagg, Kathy; Durst, Louise A.; Li, Pei; Morris, Marty; Southall, Kara L; Peterson, Leigh; Bourge, Robert C.

doi:10.1016/j.chest.2017.04.188

Cited by 17 publications

(13 citation statements)

References 11 publications

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“…Our patient also developed a hematoma at the pump pocket; hematomas/seromas have been observed previously in adult patients following pump implantation and are usually minor and easily treated with local compression/cooling or with puncture and needle aspiration. 12,14,15…”

Section: Discussionmentioning

confidence: 99%

Intravenous treprostinil via an implantable pump in pediatric pulmonary arterial hypertension

Desole¹,

Richter

Heine³

et al. 2018

Pulm. circ.

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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.

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Section: Discussionmentioning

confidence: 99%

Intravenous treprostinil via an implantable pump in pediatric pulmonary arterial hypertension

Desole¹,

Richter

Heine³

et al. 2018

Pulm. circ.

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“…Patients must apply a meticulously sterile technique to reduce infection when refilling the pump at home. A fully implantable pump decreases catheter related side effects and increases patients’ satisfaction97; however, this pump is not approved for clinical use.…”

Section: Current Treatments For Pahmentioning

confidence: 99%

Pulmonary arterial hypertension: pathogenesis and clinical management

Thenappan

Ormiston

Ryan

et al. 2018

BMJ

648

608

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Pulmonary hypertension is defined as a resting mean pulmonary artery pressure of 25 mm Hg or above. This review deals with pulmonary arterial hypertension (PAH), a type of pulmonary hypertension that primarily affects the pulmonary vasculature. In PAH, the pulmonary vasculature is dynamically obstructed by vasoconstriction, structurally obstructed by adverse vascular remodeling, and pathologically non-compliant as a result of vascular fibrosis and stiffening. Many cell types are abnormal in PAH, including vascular cells (endothelial cells, smooth muscle cells, and fibroblasts) and inflammatory cells. Progress has been made in identifying the causes of PAH and approving new drug therapies. A cancer-like increase in cell proliferation and resistance to apoptosis reflects acquired abnormalities of mitochondrial metabolism and dynamics. Mutations in the type II bone morphogenetic protein receptor (BMPR2) gene dramatically increase the risk of developing heritable PAH. Epigenetic dysregulation of DNA methylation, histone acetylation, and microRNAs also contributes to disease pathogenesis. Aberrant bone morphogenetic protein signaling and epigenetic dysregulation in PAH promote cell proliferation in part through induction of a Warburg mitochondrial-metabolic state of uncoupled glycolysis. Complex changes in cytokines (interleukins and tumor necrosis factor), cellular immunity (T lymphocytes, natural killer cells, macrophages), and autoantibodies suggest that PAH is, in part, an autoimmune, inflammatory disease. Obstructive pulmonary vascular remodeling in PAH increases right ventricular afterload causing right ventricular hypertrophy. In some patients, maladaptive changes in the right ventricle, including ischemia and fibrosis, reduce right ventricular function and cause right ventricular failure. Patients with PAH have dyspnea, reduced exercise capacity, exertional syncope, and premature death from right ventricular failure. PAH targeted therapies (prostaglandins, phosphodiesterase-5 inhibitors, endothelin receptor antagonists, and soluble guanylate cyclase stimulators), used alone or in combination, improve functional capacity and hemodynamics and reduce hospital admissions. However, these vasodilators do not target key features of PAH pathogenesis and have not been shown to reduce mortality, which remains about 50% at five years. This review summarizes the epidemiology, pathogenesis, diagnosis, and treatment of PAH.

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“…Moreover, it should be kept in mind that patients with advanced PAH represent a group at highrisk for general anesthesia. Several papers have shown that the procedure may be associated with fatal complications, especially unmanageable right-sided heart failure (11)(12)(13)(14). It is therefore crucial that the procedure is performed in centers experienced in the treatment of pulmonary hypertension and that the general anesthesia is conducted by an experienced anesthetist.…”

Section: Discussionmentioning

confidence: 99%

“…The dosage can be adjusted by using various concentrations of treprostinil loaded into the pump reservoir. So far, several papers have shown that the pump implantation is relatively safe with a low risk of periprocedural complications (11)(12)(13)(14). The long-term treatment has been also shown to be safe while complications such as local infections at the site of pump implantation, catheter obstruction associated with the risk of discontinuation of treprostinil therapy, and increased pump output associated with the risk of an increased amount of applied treprostinil due to a pump failure are relatively rare.…”

Section: Introductionmentioning

confidence: 99%

Long-term experience with implantable treprostinil pumps in pulmonary arterial hypertension

Dytrych¹,

Kuchař²,

Ambrož³

et al. 2020

BLL

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OBJECTIVES: To compare clinical parameters and quality of life in patients with pulmonary arterial hypertension (PAH) at the time of diagnosis, at the time of LenusPro pump implantation and during intravenous treptostinil treatment. METHODS: Seven patients with severe PAH treated with intravenous treptostinil via implantable LenusPro pumps were evaluated, including NYHA classifi cation, six-minute walking test, BNP and quality of life assessment using the EQ-5D-5L questionnaire before and after pump implantation. RESULTS: No signifi cant changes were observed in NYHA class and six-minute walking distance test. There was however a signifi cant improvement in the quality of life and a decrease in BNP levels. The mean EQ-5D-5L index assessed during subcutaneous treptostinil treatment was signifi cantly worse when compared to that assessed during its intravenous application (0.39 ± 0.24 vs 0.78 ± 0.28, p ˂ 0.05); the same is true about the pain/discomfort dimension. Complications occurred, namely one nonfatal pneumothorax, one nonfatal hemothorax, and one event of nonfatal treptostinil intoxication after refi lling. CONCLUSIONS: In patients who do not tolerate subcutaneous treptostinil treatment, the use of the LenusPro implantable pump results in a signifi cant improvement in quality of life with an acceptable safety profi le (Tab. 2, Fig. 2, Ref. 19).

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Totally Implantable IV Treprostinil Therapy in Pulmonary Hypertension Assessment of the Implantation Procedure

Cited by 17 publications

References 11 publications

Intravenous treprostinil via an implantable pump in pediatric pulmonary arterial hypertension

Intravenous treprostinil via an implantable pump in pediatric pulmonary arterial hypertension

Pulmonary arterial hypertension: pathogenesis and clinical management

Long-term experience with implantable treprostinil pumps in pulmonary arterial hypertension

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