Subcutaneous Treprostinil Research Articles

Dual prostacyclin infusions: A case report of a patient symptom-driven transition from high-dose intravenous epoprostenol to subcutaneous treprostinil for the treatment of pulmonary arterial hypertension.

In an effort to expedite the publication of articles, AJHP is posting manuscripts online as soon as possible after acceptance. Accepted manuscripts have been peer-reviewed and copyedited, but are posted online before technical formatting and author proofing. These manuscripts are not the final version of record and will be replaced with the final article (formatted per AJHP style and proofed by the authors) at a later time. A case of successful transition from high-dose epoprostenol to high-dose subcutaneous treprostinil for treatment of pulmonary arterial hypertension (PAH) is reported. PAH is a chronically progressive disease characterized by pulmonary artery luminal narrowing that causes increased pulmonary artery pressures leading to right ventricular failure. Parenteral prostacyclin analogues, such as epoprostenol and treprostinil, are direct vasodilators and are cornerstones of therapy for patients with severe disease that have been proven to reduce mortality and increase exercise tolerance. These agents must be administered continuously via intravenous or subcutaneous devices and are high-risk medications due to their potent vasodilatory actions. Chronic use of these medications requires constant attention from both providers and patients because of potential complications including central venous catheter infection, thromboembolism, therapy interruptions, and other undesirable consequences. This case report describes management of a 35-year-old male patient on high-dose outpatient intravenous epoprostenol (101ng/kg/min; dosing weight, 47kg) for treatment of PAH who was admitted to the hospital with a malfunctioning central venous catheter. Surrounding manipulation of the central catheter, the patient experienced an ischemic stroke that led to cognitive disability resulting in a lack of ability to manage his previously used home infusion device. The patient was successfully transitioned from intravenous epoprostenol to subcutaneous treprostinil (discharge dose, 200ng/kg/min) over 5 days by infusing both medications simultaneously and adjusting doses based upon patient-reported symptoms. This successful transition from high-dose epoprostenol to high-dose subcutaneous treprostinil demonstrates the importance in considering patient-specific factors during high-risk medication transitions, the value of a patient-directed flexible prostacyclin transition plan, and the benefit of institutional training and education in ensuring the safe use of parenteral prostacyclin analogues.

Can subcutaneous treprostinil be an alternative for treating pulmonary hypertension in patients with systemic sclerosis-related interstitial lung disease?

Can subcutaneous treprostinil be an alternative for treating pulmonary hypertension in patients with systemic sclerosis-related interstitial lung disease?

Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension.

Children with severe Group 1 pulmonary arterial hypertension (PAH) have an unpredictable response to subcutaneous treprostinil (TRE) therapy, which may be influenced by age, disease severity, or other unknown variables at time of initiation. In this retrospective single-center cohort study, we hypothesized that younger age at TRE initiation, early hemodynamic response (a decrease in pulmonary vascular resistance by ≥30% at follow-up catheterization), and less severe baseline hemodynamics (Rp:Rs < 1.1) would each be associated with better clinical outcomes. In 40 pediatric patients with Group I PAH aged 17 days-18 years treated with subcutaneous TRE, younger age (cut-off of 6-years of age, AUC 0.824) at TRE initiation was associated with superior 5-year freedom from adverse events (94% vs. 39%, p = 0.002), better WHO functional class (I or II: 88% vs. 39% p = 0.003), and better echocardiographic indices of right ventricular function at most recent follow-up. Neither early hemodynamic response nor less severe baseline hemodynamics were associated with better outcomes. Patients who did not have a significant early hemodynamic response to TRE by first follow-up catheterization were unlikely to show subsequent improvement in PVRi (1/8, 13%). These findings may help clinicians counsel families and guide clinical decision making regarding the timing of advanced therapies.

Long-Term Safety, Outcome, and Clinical Effects of Subcutaneous and Intravenous Treprostinil Treatment in Patients with Severe Chronic Pulmonary Arterial Hypertension

Background: Current guidelines recommend treatment with parenteral prostacyclin analogs in patients with severe pulmonary arterial hypertension (PAH), who have insufficient response to treatment. Real-life data are sought to help physicians in treatment decisions and clinical care of patients. Objective: This study analyzed safety, clinical effects, and long-term outcomes of subcutaneous (sc) and/or intravenous (iv) treprostinil via different pump systems in consecutive patients with PAH. Methods: Thirty-seven patients with severe progressive PAH despite dual combination therapy (20 female, mean age: 52.3 ± 15 years, mean pulmonary vascular resistance: 12.1 ± 5.1 WU) were initiated with add-on treprostinil sc and were routinely clinically assessed. Changes in clinical parameters, adverse events, and outcome were analyzed retrospectively. Results: In 24 of 37 patients, treprostinil administration was continued iv via implantation of LENUS Pro® pump after 3 ± 1.3 months, 6 patients continued with sc therapy, and 7 discontinued treatment. After 3, 6, 9, and 12 months of treprostinil treatment, patients showed a significant improvement in mean 6-min walk distance and tricuspid annular plane systolic excursion compared to baseline. In 8 of the 24 patients, iv pumps required surgical revision. During a mean follow-up of 2.82 ± 1.95 years, 12 patients died, four received lung transplantation. Transplant-free survival after 1, 2, and 3 years was 85.7%, 69.2%, and 65.3%, respectively. Conclusion: sc treprostinil as add-on to double combination treatment significantly improved exercise capacity and right heart function. In most patients, treprostinil could be continued via more tolerable iv administration approach (LENUS Pro® pump), showing reasonable overall survival with respect to the severity of PAH.

Intravenous Treprostinil in Severe Inoperable Chronic Thromboembolic Pulmonary Hypertension Using Implantable Pumps-Single-Center Experience over More Than a Decade.

The management of chronic thromboembolic pulmonary hypertension has significantly changed over the last decade with the availability of both specific therapies and interventional treatments. In parallel, implantable pumps for intravenous administration of treprostinil have broadened the spectrum of continuous prostanoid infusion. We evaluated the course of 17 consecutive patients with inoperable chronic thromboembolic pulmonary hypertension treated with treprostinil by means of an implantable infusion pump between 2011 and 2023 at our center. Complications associated with the infusion system were rare, leading to 0.4 unplanned surgical interventions during 17,160 patient days. No additional safety signals were detected, and clinical benefits achieved with subcutaneous treprostinil before pump implantation could be maintained in all patients. No catheter-related infections or thromboembolic events were observed. Implantable infusion pumps offer an attractive alternative to subcutaneous treprostinil for patients intolerant to the subcutaneous route, including those with chronic thromboembolic pulmonary hypertension.

The risk profile change in patients with severe chronic thromboembolic pulmonary hypertension treated with subcutaneous treprostinil.

Chronic thromboembolic pulmonary hypertension (CTEPH) is successfully treatable with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty, and medical therapy. Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management risk score (RRS) is able to predict long-term outcome in inoperable patients or in patients with residual PH after surgery. We performed a post hoc analysis of RRS in patients who were enrolled in the CTREPH study (NCT01416636), a randomized, double-blind clinical trial comparing high-dose and low-dose subcutaneous (SC) treprostinil in patients with severe CTEPH that was classified by an interdisciplinary CTEPH team as nonoperable, or as persistent or recurrent pulmonary hypertension after PEA. Baseline mean RRS was similar in both treatment groups (8.7 in high-dose arm vs. 8.6 in low-dose arm), but mean RRS change from baseline to Week 24 was greater in the high-dose treprostinil group than in the low-dose treprostinil group (-0.88 vs. -0.17). The difference in RRS change from baseline to Week 24 between high dose versus low dose was statistically significant with mean difference of -0.70 (95% confidence interval: -1.36 to -0.05, p = 0.0352), and was driven mainly by improvement of World Health Organization functional class and N-terminal pro-brain natriuretic peptide concentration. SC treprostinil therapy administered in standard dose had positive effect on the risk profile measured by RRS in patients with inoperable or persistent/recurrent severe CTEPH. Although our study was limited by the small sample size and post hoc nature, assessment of risk profile is of great importance to this particular patient population with very poor prognosis.

Long-Term Experience and Safety of Transitioning from Subcutaneous Treprostinil to Oral Selexipag in the High-Risk Pulmonary Hypertension Patient: A Case Report.

Long-Term Experience and Safety of Transitioning from Subcutaneous Treprostinil to Oral Selexipag in the High-Risk Pulmonary Hypertension Patient: A Case Report.

Failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patients.

Continuous subcutaneous (SubQ) treprostinil is an effective therapy for pediatric patients diagnosed with pulmonary hypertension (PH). To date, the clinical characteristics and factors associated with failure to tolerate this therapy have not been described. The purpose was to describe patient-reported factors contributing to SubQ treprostinil intolerance in pediatric patients with PH. A retrospective descriptive study was performed at 11 participating sites in the United States and Canada for patients younger than 21 years of age diagnosed with PH who failed treatment to tolerate SubQ treprostinil between January 1, 2009, and December 31, 2019. All data were summarized using descriptive statistics. Forty-one patients met the inclusion criteria. The average age at SQ treprostinil initiation, and length of treatment, was 8.6 years and 22.6 months, respectively. The average maximum dose, concentration, and rate were 95.8 ng/kg/min, 6.06 mg/mL, and 0.040 mL/h, respectively. The reasons for failure to tolerate SubQ treprostinil included intractable site pain (73.2%), frequent site changes (56.1%), severe site reactions (53.7%), infections (26.8%), and noncompliance/depression/anxiety (17.1%). Thirty-nine (95.1%) patients transitioned to a prostacyclin therapy with 23 patients transitioning to intravenous prostacyclin, 5 to inhaled prostacyclin, 5 to oral prostacyclin, and 7 to a prostacyclin receptor agonist. A subset of pediatric PH patients failed to tolerate SubQ treprostinil infusions despite advances in SubQ site maintenance and pain management strategies. Intractable site pain, frequent SubQ site changes, and severe localized skin reactions were the most common reasons for failure.

Patient Satisfaction with a Dedicated Infusion Pump for Subcutaneous Treprostinil to Treat Pulmonary Arterial Hypertension.

Background and Objectives: Parenteral prostacyclins are crucial in the pharmacological treatment of pulmonary arterial hypertension (PAH). Indeed, subcutaneous administration of treprostinil has been associated with considerable clinical and hemodynamic improvement, right-sided heart reverse remodeling, and long-term survival benefit. However, evidence on patient perceptions about handling a subcutaneous infusion pump for self-treatment administration and nurse views about training the patients are lacking. This study aimed to describe the perception of PAH patients and nurses regarding the use of the new portable I-Jet infusion pump for the self-administration of subcutaneous treprostinil, as well as its real-world training needs. Materials and Methods: The study is an open, observational, prospective, single-center, non-interventional study. Patients with PAH on stable therapy with subcutaneous treprostinil were invited to take part in the study at their start of use of the portable I-Jet infusion pump for the self-administration of treatment. Participants filled in a questionnaire to report their satisfaction with the use of the pump, as well as their compliance, confidence, convenience, preferences, technical issues, and perceptions of the training they received. Results: Thirteen patients completed the questionnaire after being on the pump for 2 months: 69% were females and the mean age was 51 years. The most frequent PAH etiologies were congenital heart disease (46.2%) and idiopathic PAH (38.4%). Most patients were either World Health Organization (WHO) functional class II (53.8%) or III (46.2%). Ten patients (76.9%) found the pump easy and convenient to live with. All patients declared themselves to be fully compliant and confident in using the pump (n = 13) at the end of the study follow-up. Ten patients (76.9%) would choose the new pump in the future. None of the patients made reference to technical issues that required additional hospital visits. Eight patients (61.6%) reported that learning how to use the I-Jet infusion pump was easy or very easy, and none considered that further training was needed. One trainer nurse was interviewed and confirmed the satisfaction of patients and the simplicity of usage and training. Conclusions: PAH patients were highly satisfied with the use of the new portable I-Jet infusion pump for self-administering subcutaneous treprostinil. Convenience and ease of use were valuable and commonly reported features. Moreover, the training requirement was simple. These preliminary findings support the routine use of the I-Jet infusion pump.

Subcutaneous use of treprostinil in pediatric pulmonary hypertension patients: A report of three cases.

Treprostinil was approved by the United States Food and Drug Administration for use in the treatment of pulmonary arterial hypertension in 2002. Intravenous or subcutaneous treprostinil is used in pulmonary arterial hypertension patients in the functional classes of II-IV to alleviate exercise-related symptoms, or in cases where epoprostenol treatment should be reduced due to side effects. In this article, we describe three pediatric cases of pulmonary arterial hypertension in whom subcutaneous treprostinil was used.

Selexipag-based triple combination therapy improves prognosis in Chinese pulmonary arterial hypertension patients.

AimSelexipag is an oral selective prostacyclin receptor agonist approved for treatment of patients with pulmonary arterial hypertension (PAH). In the present study, we aim to assess the safety and efficacy of selexipag in triple combination therapy with endothelial receptor antagonists (ERAs) and PDE5is for Chinese PAH patients.Methods and resultsA single center retrospective study was performed on group 1 PAH patients (n = 68) initiating triple combination therapy with selexipag from 1 February 2020 to 31 August 2021 in Qilu Hospital of Shandong University (Shandong, China). Adolescents, children, and PAH patients with unrepaired congenital heart disease were excluded. The French pulmonary hypertension network (FPHN) non-invasive risk assessment, echocardiogram parameters, and clinical data, including tolerability, safety, and death/hospitalization events associated with PAH, were collected. Of the 68 patients, 31 (45.6%) patients had tolerable side effects while only a single patient discontinued selexipag due to severe diarrhea. In the analysis of the efficacy set of 62 patients, the median selexipag treatment time from selexipag initiation to last risk assessment was 27 (21, 33) weeks. Compared to baseline parameters, the percentage of WHO FC III/IV decreased from 77.4% (48) to 24.2% (15) (p = 0.000), median 6-min walk distance (6MWD) increased 82 m [from 398 (318, 450) to 480 (420, 506) m; p = 0.000], and NT-proBNP levels decreased from 1,216 (329, 2,159) to 455 (134, 1,678) pg/mL (p = 0.007). Patients who improved to three low-risk criteria increased from 9.7 to 38.7%. Right ventricular diameter (RV) diameter also decreased and was accompanied by an improved tricuspid annular plane systolic excursion (TAPSE). Patients transitioning from subcutaneous treprostinil to selexipag continued to show improvements in WHO FC, 6MWD (404 ± 94 vs. 383 ± 127 m) and NT-proBNP levels (2,319 ± 2,448 vs. 2,987 ± 3,770 pg/mL). Finally, the 1-year event free survival rate was 96.7% for patients initiating the triple combination therapy within 3 years of PAH diagnosis.ConclusionTriple combination therapy with selexipag was safe and effective in Chinese PAH patients, which was confirmed by acceptable tolerability, and improved exercise capacity, right heart function, risk assessment, and prognosis.

Subcutaneous Treprostinil Improves Surgical Candidacy for Next Stage Palliation in Single Ventricle Patients With High-Risk Hemodynamics

Single ventricle (SV) patients with pulmonary vascular disease (SV-PVD) are considered poor surgical candidates for Glenn or Fontan palliation. Given limited options for Stage 1 (S1) and Stage 2 (S2) SV patients with SV-PVD, we report on the use of subcutaneous treprostinil (TRE) to treat SV-PVD in this population. This single-center, retrospective cohort study examined SV patients who were not candidates for subsequent surgical palliation due to SV-PVD and were treated with TRE. The primary outcome was ability to progress to the next surgical stage; secondary outcomes included changes in hemodynamics after TRE initiation. Between 3/2014 and 8/2021, 17 SV patients received TRE for SV-PVD: 11 after S1 and 6 after S2 (median PVR 4.1 [IQR 3.2-4.8] WU*m2 and 5.0 [IQR 1.5-6.1] WU*m2, respectively). Nine of 11 (82%) S1 progressed to S2, and 2 (18%) underwent heart transplant (HTx). Three of 6 (50%) S2 progressed to Fontan, 1 underwent HTx and 2 are awaiting Fontan on TRE. TRE significantly decreased PVR in S1 patients with median post-treatment PVR of 2.0 (IQR 1.5-2.6) WU*m2. TRE can allow for further surgical palliation in select pre-Fontan patients with SV-PVD, obviating the need for HTx. Improvement in PVR was significant in S1 patients and persisted beyond discontinuation of therapy for most patients.

Long-term effect of subcutaneous treprostinil in patients with pulmonary hypertension: rationale and design of the phase IV, multicentre, observational TREPAR-HP study

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Pulmonary hypertension (PH) is a chronic, progressive condition with high morbidity and mortality due to right heart (RH) failure. Prognosis depends on RH adaptability and remodelling in response to increased pulmonary arterial pressure. There is little information regarding risk variables and prognostic factors in the Argentinian population, current risk scores have not been validated, and its impact on free-event survival is still unknown. Prostacyclin analogues are the first-line treatment for high-risk patients. However, there is limited evidence on its clinical benefits, long-term effects, and impact on RH remodelling in the Argentinian population.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; The study was designed as a national, multicentre, prospective, observational, phase IV study including 100 patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension, treated with subcutaneous treprostinil and followed up for 48 months. The study will be conducted in parallel with the patients' standard management and treatment in each centre. The primary objective is to assess the long-term improvement or reversal of RH remodelling (RHRR) parameters obtained by echocardiography. Secondary objectives include the evaluation of the prognostic value of RHRR parameters and the rate of clinical events, the treatment effect in terms of pulmonary vascular resistance, right ventricle systolic function, morbidity and all-cause mortality, quality of life, and safety and tolerability.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;This study will help to determine the clinical benefits, long-term effects, and impact on RH remodelling of prostacyclin analogues in Argentina.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Trial registration:&lt;/strong&gt; The trial was registered at Registro Nacional de Investigaciones en Salud (RENIS) in Argentina (https://sisa.msal.gov.ar/sisa/#sisa; registration number IS003303).&lt;/p&gt;

Comparison of Healthcare Encounters and Drug Persistence in Patients With Pulmonary Arterial Hypertension Receiving Oral Selexipag, Inhaled Iloprost, or Parenteral Treprostinil: A Retrospective Database Analysis

Background: Agents targeting the prostacyclin (PGI2) pathway are important in managing pulmonary arterial hypertension (PAH). No head-to-head clinical trials have compared outcomes between the 3 different PGI2-pathway drugs most commonly available in countries with advanced healthcare: oral selexipag, inhaled iloprost, and parenteral (subcutaneous or intravenous) treprostinil. Objectives: To conduct retrospective database analyses to describe characteristics of patients with PAH initiating therapy with these agents and compare the rate and risk of healthcare facility encounters and drug persistence. Methods: Data were obtained from the Optum™ Clinformatics® Data Mart and Truven™ Health Analytics® MarketScan® Commercial Claims and Encounters databases from July 1, 2008, to September 30, 2020 (Optum™), or October 31, 2020 (Truven™). Patients were categorized into index-drug cohorts based on first pharmacy claims for selexipag, inhaled iloprost, or parenteral treprostinil. Eligible patients were ≥18 years of age with ≥1 ICD-9-CM or ICD-10-CM diagnosis code indicating pulmonary hypertension and no diagnosis code suggesting Group 3–5 pulmonary hypertension. Rates of hospitalization (inpatient admissions), emergency room visits, or outpatient visits per person-year were calculated. Drug persistence was measured as time to discontinuation of index drug. Multivariable analyses were performed to compare outcomes with selexipag vs inhaled iloprost and parenteral treprostinil, adjusting for baseline characteristics using inverse probability of treatment weighting. Results: Overall, 583 patients were included in the Optum™ sample and 482 in the Truven™ sample. Mean (SD) age was 61.7 (14.5) and 49.3 (11.3) years, respectively; 74.4% and 75.7% of patients, respectively, were women. In the pooled samples, after adjustment for baseline characteristics, selexipag had a lower risk than inhaled iloprost or parenteral treprostinil for hospitalization (relative rate ratio [95% CI], 0.40 [0.22, 0.75], and 0.26 [0.17, 0.39]) and outpatient visits (0.66 [0.56, 0.78] and 0.76 [0.66, 0.88]). Trends toward lower risk of emergency room visits did not attain statistical significance. Drug discontinuation risk was 16% and 36% lower with selexipag vs parenteral treprostinil and inhaled iloprost, respectively. Conclusions: In real-world use, selexipag appears to be associated with lower rates of hospitalization and outpatient visits than inhaled iloprost or parenteral treprostinil. Further research is required to identify factors underlying these differences.

Comparison of Healthcare Encounters and Drug Persistence in Patients With Pulmonary Arterial Hypertension Receiving Oral Selexipag, Inhaled Iloprost, or Parenteral Treprostinil: A Retrospective Database Analysis.

Background: Agents targeting the prostacyclin (PGI2) pathway are important in managing pulmonary arterial hypertension (PAH). No head-to-head clinical trials have compared outcomes between the 3 different PGI2-pathway drugs most commonly available in countries with advanced healthcare: oral selexipag, inhaled iloprost, and parenteral (subcutaneous or intravenous) treprostinil.Objectives: To conduct retrospective database analyses to describe characteristics of patients with PAH initiating therapy with these agents and compare the rate and risk of healthcare facility encounters and drug persistence.Methods: Data were obtained from the Optum™ Clinformatics® Data Mart and Truven™ Health Analytics® MarketScan® Commercial Claims and Encounters databases from July 1, 2008, to September 30, 2020 (Optum™), or October 31, 2020 (Truven™). Patients were categorized into index-drug cohorts based on first pharmacy claims for selexipag, inhaled iloprost, or parenteral treprostinil. Eligible patients were ≥18 years of age with ≥1 ICD-9-CM or ICD-10-CM diagnosis code indicating pulmonary hypertension and no diagnosis code suggesting Group 3–5 pulmonary hypertension. Rates of hospitalization (inpatient admissions), emergency room visits, or outpatient visits per person-year were calculated. Drug persistence was measured as time to discontinuation of index drug. Multivariable analyses were performed to compare outcomes with selexipag vs inhaled iloprost and parenteral treprostinil, adjusting for baseline characteristics using inverse probability of treatment weighting.Results: Overall, 583 patients were included in the Optum™ sample and 482 in the Truven™ sample. Mean (SD) age was 61.7 (14.5) and 49.3 (11.3) years, respectively; 74.4% and 75.7% of patients, respectively, were women. In the pooled samples, after adjustment for baseline characteristics, selexipag had a lower risk than inhaled iloprost or parenteral treprostinil for hospitalization (relative rate ratio [95% CI], 0.40 [0.22, 0.75], and 0.26 [0.17, 0.39]) and outpatient visits (0.66 [0.56, 0.78] and 0.76 [0.66, 0.88]). Trends toward lower risk of emergency room visits did not attain statistical significance. Drug discontinuation risk was 16% and 36% lower with selexipag vs parenteral treprostinil and inhaled iloprost, respectively.Conclusions: In real-world use, selexipag appears to be associated with lower rates of hospitalization and outpatient visits than inhaled iloprost or parenteral treprostinil. Further research is required to identify factors underlying these differences.

Meeting abstracts from the 15th international conference on neonatal and childhood pulmonary vascular disease

Meeting abstracts from the 15th international conference on neonatal and childhood pulmonary vascular disease

Transition from Parenteral to Oral Prostacyclin Agents in Pulmonary Arterial Hypertension in Ambulatory Care Setting: A Tertiary Care Institutional Experience

<h3>Purpose</h3> Prostacyclins are used to treat advanced pulmonary arterial hypertension as continuous intravenous or subcutaneous infusions. In the right clinical setting, parenteral Prostacyclins can be transitioned to newer oral Prostacyclins. There is a dearth of published guidance for this transition and is limited to inpatient setting. We present our experience with transitioning patients with WHO group 1 pulmonary arterial hypertension from parenteral Treprostinil to oral Treprostinil in the outpatient setting. <h3>Methods</h3> 6 patients with WHO group 1 pulmonary arterial hypertension on stable doses of intravenous or subcutaneous Treprostinil for at least 6 months were transitioned to oral Treprostinil as per published protocol albeit at a slower rate of transition at 6 ng/kg/min per day <h3>Results</h3> All the 6 patients were successfully transitioned from Parenteral Treprostinil to oral Treprostinil and 4 remain on oral therapy for 1- 4 years. The reasons for transition include complications of central line, side effects related to parenteral Prostacyclins and personal preference. Two patients deteriorated over 6-12 months after the transition requiring transition back to parenteral Treprostinil. No deterioration in PAH was noted during the transition. High 6 MWD, High WHO FC (1/2) and normal RV function on parenteral Prostacyclins are associated with successful results. <h3>Conclusion</h3> In the right clinical setting, patients can be successfully transitioned from parenteral Treprostinil to oral Treprostinil and this can be accomplished safely in the outpatient setting.

Clinical impact of subcutaneous treprostinil in trisomy 21 patient with pulmonary arterial hypertension associated with CHD.

Subcutaneous treprostinil is commonly used to improve idiopathic pulmonary arterial hypertension in children. However, its effectiveness has not been reported in trisomy 21. We report the case of 9-year-old boy in trisomy 21 with CHD-pulmonary artery hypertension after surgical correction of CHD. Haemodynamics and exercise capacity dramatically improved with a transition from oral selexipag to subcutaneous treprostinil.

Real-world evidence of subcutaneous treprostinil use in pulmonary arterial hypertension in Argentina.

Pulmonary arterial hypertension is a progressive haemodynamic disease with high morbidity and mortality. Of the different treatments available, the prostacyclin analogues are the drugs of choice for high-risk patients, with treprostinil being the most commonly used drug in Argentina. The objective of this study is to perform a retrospective evaluation of the efficacy and safety of subcutaneous treprostinil in regular clinical practice in Argentina in 51 patients with pulmonary arterial hypertension after 12 months of follow-up. The results showed that treatment with subcutaneous treprostinil is associated with a significant improvement in different clinical efficacy parameters: 65% reduction in advanced functional class (p < 0.0001), 130-m increase in the 6-min walk test (p < 0.0001), 65% reduction in the pro B-type natriuretic peptide value (-531 pg/dL; p < 0.0001), significant reduction of 15.7% in pulmonary vascular resistance [-1.3 wood units (WU); p < 0.0001], improved cardiac index with an increase of 16.7% (+0.4 L/min/m2; p = 0.002), as well as a high survival rate (92%) and a 44% incidence of combined events (mortality, heart failure, syncope and/or lung transplantation), without a significant increase in previously reported adverse events. The risk stratification evaluation according to ESC/ERS guidelines showed a significant decrease in the proportion of patients at high risk after the treatment period (p = 0.004). These real-world results corroborate the efficacy and safety of subcutaneous treprostinil, even at high doses, and open up the possibility of improving its current use in clinical practice as a first-line therapy, especially in high-risk patient profiles.

Transition from Oral Selexipag to Subcutaneous Treprostinil in a Patient with Idiopathic Pulmonary Arterial Hypertension: An Eight-Day Protocol

Transition from Oral Selexipag to Subcutaneous Treprostinil in a Patient with Idiopathic Pulmonary Arterial Hypertension: An Eight-Day Protocol