P3671Selexipag dosing and titration in the first 500 patients enrolled in SPHERE (SelexiPag: tHe UsErs dRug rEgistry)

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
V McLaughlin ◽  
N H Kim ◽  
A R Hemnes ◽  
K B Highland ◽  
K M Chin ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Adverse Events ◽  
Maintenance Dose ◽  
Receptor Agonist ◽  
Maximum Dose ◽  
Functional Class ◽  
Class Iii ◽  
Prostacyclin Receptor ◽  
Drug Registry ◽  
Group 1

Abstract Introduction SPHERE is an ongoing US-based, multicentre, prospective, registry collecting data on use of the oral selective IP prostacyclin receptor agonist selexipag in real-world settings. Here, we report selexipag dosing and titration in the first 500 patients. Methods SPHERE, initiated in November 2016, will enrol 800 patients newly initiated on or already treated with selexipag at enrolment who have a documented titration regimen. Patients are followed for up to 18 months. Patients were considered “newly initiated” on selexipag if they were enrolled in SPHERE ≤60 days after starting selexipag and were considered “previously initiated” if they enrolled >60 days after starting selexipag. The highest dose is the maximum dose reached during up-titration within 6 months since initiation. Selexipag “maintenance dose” is defined as the first dose received for ≥14 days without interruption or change; “titration speed” is defined as the highest dose reached within the first 6 months after initiation divided by the time (in weeks) to reach it. Results The data cut-off for this analysis was December 20, 2018. Most patients had Group 1 pulmonary hypertension (PH) (95.4%), which was primarily idiopathic (49.6%) or connective tissue disease associated (26.0%). At selexipag initiation 49.8% of patients had functional class III symptoms. At the time of selexipag initiation, 19.2% of patients were on PH therapy containing a prostacyclin pathway agent (PPA) (8.5% with a parenteral PPA). The median maintenance dose of selexipag was 1200 μg BID (IQR: 800–1600 μg BID) and the median time to reach it was 8.1 wks (IQR: 5.3–11.0 wks). Low (≤400 μg BID), medium (600–1000 μg BID), and high (≥1200 μg BID) maintenance doses were attained by 15.1%, 30.8%, and 49.5% of patients, respectively (and in 23.2%, 31.2%, and 36.2%, respectively, in GRIPHON). The median titration speed was 175 μg BID/wk (IQR: 110.5–195.3 μg BID/wk), slower than the protocol-outlined 200 μg BID/wk in GRIPHON. In SPHERE, most patients titrated at speeds <200 μg BID/wk, regardless of whether they were newly (175 μg BID/wk; IQR 118.6, 195.3) or previously (175 μg BID/wk; IQR 109.8, 195.3) initiated. As expected, more patients discontinued due to adverse events in the newly (29.0%) versus previously (14.1%) initiated groups. The most common adverse events leading to selexipag discontinuation were worsening pulmonary hypertension (2.2%), headache (2.0%), myalgia (1.4%), and nausea (1.0%). Conclusion The median maintenance selexipag dose in SPHERE was 1200 μg BID. While the median titration speed was 175 μg BID/wk, there was marked variation and the vast majority of patients titrated slower than 200 μg BID/wk. No new safety signals were observed. Acknowledgement/Funding Actelion Pharmaceuticals US, Inc.

scholarly journals Characterization of a Cohort of Patients with Chronic Thromboembolic Pulmonary Hypertension from Northeastern Colombia (REHINO Study)

2021 ◽  
Vol 1 (2) ◽  
pp. 105-113
Author(s):  
Javier Enrique Fajardo-Rivero ◽  
Melissa Mogollón ◽  
Diego Fernando García-Bohórquez ◽  
Andrés Villabona-Rueda ◽  
Tania Mendoza-Herrera ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Thrombotic Event ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pharmacological Therapy ◽  
Functional Class ◽  
World Health ◽  
Class Iii ◽  
European Respiratory Society ◽  
Cross Sectional ◽  
Number Of Patients

Chronic thromboembolic disease (CTEPH) is one of the causes for developing pulmonary hypertension (PH). PH is characterized by an increase in pulmonary vascular pressure and resistance, ultimately leading to chronic overload. This study describes the clinical, functional, and hemodynamic characteristics as well as the established treatment strategy for a cohort of patients diagnosed with CTEPH in Bucaramanga, Colombia. In Colombia, PH is considered as an orphan disease with limited epidemiological data. We aim to provide useful information in order to help guide future clinical decisions for PH treatment and prevention. We conducted a cross-sectional study, obtaining clinical data from patients under follow-up, over 18 years of age, with hemodynamic confirmation of CTEPH in two pulmonary outpatient centers in Bucaramanga, Colombia between 2012 and 2018. 35 patients with diagnosis of CTEPH were included. Mean age was 52.3 ± 17.9 years. The mean time between the onset of symptoms to diagnosis was 14 months. 71% had a previous thrombotic event and 69% had functional class III and IV according to the world health organization (WHO) criteria. Most of the patients were classified as at high risk of mortality according to the European Society of Cardiology (ESC) and the European Respiratory Society (ERS/ESC) criteria and 60% were referred to undergo thromboendarterectomy. Most of the patients were under monotherapy treatment with Bosentan, the most prescribed medication in both monotherapy and dual therapy. This study identified a high number of patients in advanced stages of CETPH due to late diagnosis, related to health care limitations. This resulted in worse prognosis and quality of life. In addition, low adherence to non-pharmacological interventions was evidenced in patients who were not candidates for thromboendarterectomy despite the onset of pharmacological therapy.

Arterial embolisation and coiling for high-output heart failure and pulmonary hypertension ınduced by hepatic arteriovenous fistula in a patient with hereditary hemorrhagic telengiectasia

Open Medicine ◽  
2009 ◽  
Vol 4 (3) ◽  
pp. 369-373
Author(s):  
Sadık Açikgöz ◽  
Gülten Taçoy ◽  
Baran Önal ◽  
Beytullah Yıldırım ◽  
Atiye Çengel
Keyword(s):  
Heart Failure ◽  
New York ◽  
Pulmonary Hypertension ◽  
Arteriovenous Fistula ◽  
Arteriovenous Malformations ◽  
Nyha Class ◽  
Severe Heart Failure ◽  
Heart Association ◽  
Functional Class ◽  
Class Iii

AbstractHereditary hemorrhagic telangiectasia (HHT) is a genetic vascular disorder characterised by epistaxis, telangiectases, and visceral arteriovenous malformations. Hyperdynamic blood flow associated with arteriovenous malformations may lead to pulmonary hypertension, global heart failure, and valvular insufficiencies. We report a patient who had HHT with severe heart failure (New York Heart Association [NYHA] class III-IV) and pulmonary hypertension caused by an hepatic arteriovenous fistula. After successful transarterial embolisation of the right branch of the hepatic artery with polyvinyl alcohol (PVA) particles and coils, 4 to 7 mm in size, the patient was discharged with functional class II (NYHA) heart failure.

Selexipag use for paediatric pulmonary hypertension: a single centre report focussed on congenital heart disease patients

2021 ◽  
pp. 1-3
Author(s):  
Alvaro Lafuente-Romero ◽  
Alejandro Rodriguez Ogando
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Poor Prognosis ◽  
Receptor Agonist ◽  
Congenital Heart ◽  
Complex Disease ◽  
Pulmonary Artery Hypertension ◽  
Single Centre ◽  
Prostacyclin Receptor

Abstract Pulmonary hypertension is a rare and complex disease with poor prognosis. Paediatric cases are infrequent and usually associated with congenital heart disease. Management is problematical due to the limited therapy available and poor evidence of efficacy. Recently a new medication, selexipag (UptraviR), a prostacyclin receptor agonist, has been approved for the treatment of pulmonary artery hypertension in adults. We report our experience using selexipag in four paediatric patients with pulmonary hypertension associated with congenital heart disease.

scholarly journals Macitentan in pulmonary hypertension due to left ventricular dysfunction

2018 ◽  
Vol 51 (2) ◽  
pp. 1701886 ◽  
Author(s):  
Jean-Luc Vachiéry ◽  
Marion Delcroix ◽  
Hikmet Al-Hiti ◽  
Michela Efficace ◽  
Martin Hutyra ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Adverse Events ◽  
Diastolic Pressure ◽  
Heart Association ◽  
Left Ventricular ◽  
Functional Class ◽  
Fluid Retention ◽  
Right Atrial ◽  
Serious Adverse Events ◽  
End Points

The MELODY-1 study evaluated macitentan for pulmonary hypertension because of left heart disease (PH-LHD) in patients with combined post- and pre-capillary PH.63 patients with PH-LHD and diastolic pressure gradient ≥7 mmHg and pulmonary vascular resistance (PVR) >3WU were randomised to macitentan 10 mg (n=31) or placebo (n=32) for 12 weeks. The main end-point assessed a composite of significant fluid retention (weight gain ≥5% or ≥5 kg because of fluid overload or parenteral diuretic administration) or worsening in New York Heart Association functional class from baseline to end of treatment. Exploratory end-points included changes in N-terminal pro-brain natriuretic peptide (NT-proBNP) and haemodynamics at week 12.Seven macitentan-treated and four placebo-treated patients experienced significant fluid retention/worsening functional class; treatment difference, 10.08% (95% CI −15.07–33.26; p=0.34). The difference, driven by the fluid retention component, was apparent within the first month. At week 12, versus placebo, the macitentan group showed no change in PVR, mean right atrial pressure or pulmonary arterial wedge pressure; a non-significant increase in cardiac index (treatment effect 0.4 (95% CI 0.1–0.7) L·min−1·m−2) and decrease in NT-proBNP (0.77 (0.55–1.08)) was observed. Adverse events and serious adverse events were numerically more frequent with macitentan versus placebo.Macitentan-treated patients were quantitatively more likely to experience significant fluid retention versus placebo. Macitentan resulted in no significant changes in any exploratory end-points.

scholarly journals EXPRESS: A multicenter retrospective study of patients with pulmonary hypertension transitioned from inhaled to oral treprostinil

2021 ◽  
pp. 204589402199820
Author(s):  
Diane L Zwicke ◽  
Ricardo Restrepo-Jaramillo ◽  
Hassan Alnuaimat ◽  
Kathryn Gordon ◽  
Meredith Broderick ◽  
...  
Keyword(s):  
New York ◽  
Pulmonary Hypertension ◽  
Retrospective Chart Review ◽  
Heart Association ◽  
Oral Formulation ◽  
Functional Class ◽  
World Health ◽  
Class Iii ◽  
New York Heart Association ◽  
Health Organization

Oral treprostinil has recently been shown to delay disease progression in patients with pulmonary arterial hypertension (PAH) in a long-term outcomes study. The potential advantages of an oral formulation have resulted in patients transitioning from inhaled to oral treprostinil. The current study reports a retrospective analysis of patients who transitioned from treatment with inhaled to oral treprostinil. A multicenter retrospective chart review was conducted for 30 patients with pulmonary hypertension that transitioned from inhaled to oral treprostinil. Data were collected from inhaled treprostinil initiation and patients were followed until discontinuation of oral treprostinil or the end of the observation period. Persistence was calculated using Kaplan-Meier estimates. Prior to transition to oral treprostinil, patients had received inhaled treprostinil for a median of 643 (IQR: 322, 991) days and 52% of patients were New York Heart Association (NYHA)/World Health Organization (WHO) Functional Class III. For patients that cross titrated between formulations, the median time to complete the cross titration was 24 (IQR: 1, 57) days. At 16- and 24-weeks post transition, oral treprostinil persistence was 86% and 76%, respectively. Persistence was 59% at 52 weeks post transition. Clinical stability for the majority of patients at first follow-up post transition was suggested based on available NYHA/WHO Functional Classification. Transitions from inhaled to oral treprostinil appeared safe and tolerable in the short-term. Additional prospective studies are needed to fully evaluate the safety and efficacy of transitions from inhaled to oral treprostinil.

scholarly journals An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan

2017 ◽  
Vol 26 (143) ◽  
pp. 160121 ◽  
Author(s):  
Henning Gall ◽  
Marius M. Hoeper ◽  
Manuel J. Richter ◽  
William Cacheris ◽  
Barbara Hinzmann ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Heart Association ◽  
Epidemiological Data ◽  
Epidemiological Studies ◽  
Functional Class ◽  
Class Iii ◽  
Projection Model ◽  
The Usa ◽  
Thromboembolic Pulmonary Hypertension

Epidemiological data for chronic thromboembolic pulmonary hypertension (CTEPH) are limited and there are conflicting reports regarding its pathogenesis.A literature review was conducted to identify CTEPH epidemiological data up to June 2014. Data were analysed to provide estimates of the incidence of CTEPH in the USA, Europe and Japan. An epidemiological projection model derived country-specific estimates of future incidence and diagnosis rates of CTEPH.Overall, 25 publications and 14 databases provided quantitative epidemiological data. In the USA and Europe, the crude annual incidence of diagnosed pulmonary embolism and crude annual full (i.e.diagnosed and undiagnosed) incidence of CTEPH were 66–104 and 3–5 cases per 100 000 population, respectively, while in Japan these rates were lower at 6.7 and 1.9 per 100 000 population, respectively. In 2013, 7–29% of CTEPH cases in Europe and the USA were diagnosed, and the majority of patients were in New York Heart Association functional class III/IV at diagnosis. The projection model indicated that incidence of CTEPH will continue to increase over the next decade.These data suggest that CTEPH is underdiagnosed and undertreated, and there is an urgent need to increase awareness of CTEPH. High-quality epidemiological studies are required to increase understanding of CTEPH.

scholarly journals Drug Encapsulated Nanomaterials as Carriers Used in Cardiology Field

2020 ◽  
Vol 71 (2) ◽  
pp. 413-417
Author(s):  
Alina Costina Luca ◽  
Lucian Eva ◽  
Letitia Doina Duceac ◽  
Geta Mitrea ◽  
Constantin Marcu ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Exercise Tolerance ◽  
Ethyl Cellulose ◽  
Progressive Increase ◽  
Functional Class ◽  
Class Iii ◽  
Evaporation Technique ◽  
Pulmonary Arterial ◽  
Optimum Treatment Strategy ◽  
Incorporation Efficiency

Pulmonary arterial hypertension is a disorder of high mortality being described by a progressive increase in pulmonary vascular strength leading to right ventricular damage and death. Endotelin-1 (ET-1) has an important pathogenic fuction in pulmonary hypertension. Bosentan is an oral ET-1 receptor antagonist that proved to be efficient at exercise tolerance in patients with pulmonary hypertension in functional class III and IV. The early trial registered for functional class II patients and evaluated hemodynamic at 6 month. Considerable side effects of bosentan comprise anemia, edema, and transaminase raise. Further studies were developed to determine optimum treatment strategy by eliminating adverse effects of bosentan. The major aim of this work was to prepare bosentan loaded nanoparticles by solvent evaporation technique for a sustained release of the drug by using ethyl cellulose as polymer. Drug and polymer were dissolved in ethanol at different drug-polymer ratios i.e. 1:2 and 1:3. Among the two formulations, 1:3 formulations were appreciated as the best formulation with better drug content (97.5%) and incorporation efficiency (95.5%).

scholarly journals Prostacyclin receptor agonist selexipag in a patient with high-risk idiopathic pulmonary arterial hypertension: a case report

2021 ◽  
Vol 20 (5) ◽  
pp. 3010
Author(s):  
E. A. Rezukhina ◽  
O. V. Rodnenkov ◽  
T. V. Martynyuk
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Receptor Agonist ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
World Health ◽  
Class Iii ◽  
National Medical ◽  
Prostacyclin Receptor ◽  
Pulmonary Arterial

We present a 48-year-old patient with World Health Organization class III idiopathic pulmonary arterial hypertension (IPAH), taking specific therapy with macitentan 10 mg a day, who was readmitted to the National Medical Research Center of Cardiology due to increase in exercise dyspnea and decrease in effort tolerance. According to a comprehensive examination, single factors of high risk and unfavorable prognosis were identified. Due to systemic hypotension when using inhaled iloprost, selexipag was added to therapy. According to control hospitalization, 8-month selexipag therapy improved the patient's condition, as well as high risk factors were not revealed. Selexipag is a selective oral prostacyclin receptor agonist recommended for longterm IPAH therapy in adult patients.

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