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.

scholarly journals Upfront triple oral combination therapy including selexipag in a high-risk patient with idiopathic pulmonary arterial hypertension: a case report

2020 ◽  
Vol 4 (5) ◽  
pp. 1-5
Author(s):  
Serena Rossi ◽  
Carla Pietrangelo ◽  
Sante Donato Pierdomenico ◽  
Livio Giuliani
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
High Risk Patient ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
World Health ◽  
Controlled Clinical Trial ◽  
Pulmonary Arterial

Abstract Background  Selexipag is an oral selective prostacyclin IP receptor agonist approved in patients with low- and intermediate-risk pulmonary hypertension (PH); evidence in patients at high risk is lacking. Case summary  A 42-year-old woman with worsening dyspnoea (World Health Organization functional class III–IV) and suspected PH at echocardiographic examination was evaluated in our Pulmonary Hypertension Centre. Right heart catheterization showed pre-capillary PH with reduced cardiac index and increased pulmonary vascular resistance. High-resolution computed tomography excluded parenchymal lung disease and ventilation/perfusion (V/Q) lung scan was negative for mismatched perfusion defects so the conclusive diagnosis was high-risk idiopathic pulmonary arterial hypertension (PAH). The patient refused an initial combination therapy including a parenteral prostacyclin analogue (PCA) in accordance with the ESC/ERS guidelines, so an off-label triple oral combination therapy including a phosphodiesterase-5 inhibitor, an endothelin receptor antagonist, and selexipag was started. At 3- and 6-month follow-up we found a clinical and haemodynamic improvement, so the patient was reclassified as low risk. Her clinical condition is currently stable. Discussion  Despite the benefit of parenteral PCAs in high-risk PAH, low adherence to treatment may be explained by adverse side effects related to the intravenous route of administration. Given the potential effect seen in our patient, upfront triple oral combination therapy in PAH high-risk patients should be further evaluated in a controlled clinical trial.

scholarly journals Triple combination therapy with macitentan, riociguat, and selexipag in a patient with idiopathic pulmonary arterial hypertension (functional class III)

Kardiologiia ◽  
2021 ◽  
Vol 61 (10) ◽  
pp. 104-107
Author(s):  
A. A. Proshkina ◽  
N. A. Tsareva ◽  
G. V. Nekludova ◽  
S. N. Avdeev
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Clinical Manifestations ◽  
Functional Class ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Triple Combination ◽  
Class Iii ◽  
Triple Combination Therapy ◽  
Pulmonary Arterial

The article presents a clinical case of successful triple combination therapy in a female patient with functional class III idiopathic pulmonary arterial hypertension. Supplementing the previous macitentan and riociguat treatment with selexipag reduced the severity of clinical manifestations of pulmonary hypertension. Also, the treatment efficacy was demonstrated by improvement of laboratory and instrumental indexes. Time-related changes were evaluated at 3 months after initiation of the selexipag treatment.

Single-center prognostic validation of the risk assessment of the 2015 ESC/ERS guidelines in patients with pulmonary arterial hypertension in Japan

2020 ◽  
Vol 98 (9) ◽  
pp. 653-658 ◽  
Author(s):  
Ryo Imai ◽  
Shiro Adachi ◽  
Masahiro Yoshida ◽  
Shigetake Shimokata ◽  
Yoshihisa Nakano ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Risk Group ◽  
Low Risk ◽  
World Health ◽  
Right Atrial ◽  
Walking Distance ◽  
Pulmonary Arterial

The 2015 European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension include a multidimensional risk assessment for patients with pulmonary arterial hypertension (PAH). However, prognostic validations of this risk assessment are limited, especially outside Europe. Here, we validated the risk assessment strategy in PAH patients in our institution in Japan. Eighty consecutive PAH patients who underwent right heart catheterization between November 2006 and December 2018 were analyzed. Patients were classified as low, intermediate, or high risk by using a simplified version of the risk assessment that included seven variables: World Health Organization functional class, 6-min walking distance, peak oxygen consumption, brain natriuretic peptide, right atrial pressure, mixed venous oxygen saturation, and cardiac index. The high-risk group showed significantly higher mortality than the low- or intermediate-risk group at baseline (P < 0.001 for both comparisons), and the mortalities in the intermediate- and low-risk groups were both low (P = 0.989). At follow-up, patients who improved to or maintained a low-risk status showed better survival than those who did not (P = 0.041). Our data suggest that this risk assessment can predict higher mortality risk and long-term survival in PAH patients in Japan.

scholarly journals The Modified Borg Dyspnea Scale does not predict hospitalization in pulmonary arterial hypertension

2017 ◽  
Vol 7 (2) ◽  
pp. 384-390 ◽  
Author(s):  
Debasree Banerjee ◽  
Jane Kamuren ◽  
Grayson L. Baird ◽  
Amy Palmisciano ◽  
Ipsita Krishnan ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
World Health ◽  
Pulmonary Vascular Disease ◽  
Common Symptom ◽  
Class Iii ◽  
Patient Reported ◽  
Pulmonary Arterial ◽  
Minute Walk

Background Breathlessness is the most common symptom reported by patients with pulmonary arterial hypertension (PAH). The Modified Borg Dyspnea Scale (MBS) is routinely obtained during the six-minute walk test in the assessment of PAH patients, but it is not known whether the MBS predicts clinical outcomes such as hospitalizations in PAH. Methods We performed a retrospective study of World Health Organization (WHO) Group 1 PAH patients followed at our center. The dates of the first three MBS and hospitalizations that occurred within three months of a documented MBS were collected. Marginal Cox hazard regression modeling was used to assess for a relationship between MBS and all-cause as well as PAH-related hospitalization. Results A total of 50 patients were included; most (92%) were functional class III/IV, 44% and 65% were treatment-naïve prior to their first MBS and hospitalization, respectively. The first recorded MBS was inversely correlated with the first recorded six-minute walk distance (6MWD) (r = –0.41, P < 0.01) but did not track with WHO functional class (r = 0.07, P = 0.63). MBS did not predict all-cause (hazard ratio [HR], 0.91; 95% confidence interval [CI], 0.76–1.08; P = 0.28) or PAH-related hospitalization (HR, 1.04; 95% CI, 0.89–1.23; P = 0.61), though there was a strong relationship between 6MWD and PAH-related hospitalization ( P = 0.01). These findings persisted after multivariable adjustment. Conclusions Breathlessness as assessed by MBS does not predict all-cause or PAH-related hospitalization. Robust and validated patient-reported outcomes are needed in pulmonary vascular disease.

Role of vascular remodeling markers in the development of osteoporosis in idiopathic pulmonary arterial hypertension

2016 ◽  
Vol 88 (9) ◽  
pp. 65-70
Author(s):  
V A Nevzorova ◽  
E A Kochetkova ◽  
L G Ugay ◽  
Yu V Maistrovskaya ◽  
E A Khludeeva
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Cardiac Index ◽  
Arterial Hypertension ◽  
Healthy Volunteers ◽  
Vascular Remodeling ◽  
Serum Levels ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Pulmonary Arterial

Aim. To define the role of circulating biomarkers for the metabolism of collagen and intercellular substance and vascular remodeling in the development of osteoporosis (OP) in idiopathic pulmonary arterial hypertension (IPAH). Materials and methods. Functional hemodynamic parameters, bone mineral density (BMD) in the lumbar spine and femoral neck and the serum levels of matrix metalloproteinase-9 (MMP-9), tissue inhibitor of metalloproteinase-1 (TIMP-1), MMP-9/TIMP-1 complex, C-terminal telopeptide of collagen type 1 (CITP), and endothelin-1 (ET-1) were determined in 27 high-risk IPAH patients and 30 healthy volunteers. Results. OP in IPAH was detected in 50% of the examinees. The serum levels of CITP, MMP-9, TIMP-1, and ET-1 proved to be higher in the high-risk IPAH patients than in the healthy volunteers. There was a direct correlation between BMD and six-minute walk test and an inverse correlation with total pulmonary vascular resistance (TPVR). Serum TMIP-1 levels correlated with cardiac index and TPVR; ET-1 concentrations were directly related to pulmonary artery systolic pressure, cardiac index, and TPVR. Inverse relationships were found between BMD and circulating CITP, MMP-9, TMIP-1, MMP-9/TMIP-1, and ET-1. At the same time, there was only a tendency towards a positive correlation between serum CITP and ET-1 concentrations. Conclusion. The results of the investigation confirm that endothelin system dysregulation plays a leading role in the development of persistent hemodynamic disorders in high-risk IPAH and suggest that it is involved in the development of osteopenic syndrome. Enhanced ET-1 secretion initiates bone loss possibly via activation of connective tissue matrix destruction.

scholarly journals Selexipag: An Oral and Selective IP Prostacyclin Receptor Agonist for the Treatment of Pulmonary Arterial Hypertension

2015 ◽  
Vol 58 (18) ◽  
pp. 7128-7137 ◽  
Author(s):  
Tetsuo Asaki ◽  
Keiichi Kuwano ◽  
Keith Morrison ◽  
John Gatfield ◽  
Taisuke Hamamoto ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Receptor Agonist ◽  
Prostacyclin Receptor ◽  
Pulmonary Arterial
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