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 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.

Temporal trends in pulmonary arterial hypertension: Results from the COMPERA registry

2021 ◽  
pp. 2102024
Author(s):  
Marius M. Hoeper ◽  
Christine Pausch ◽  
Ekkehard Grünig ◽  
Gerd Staehler ◽  
Doerte Huscher ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Temporal Trends ◽  
Survival Rates ◽  
Term Survival ◽  
Pulmonary Arterial ◽  
Early Combination Therapy

BackgroundSince 2015, the European pulmonary hypertension guidelines recommend the use of combination therapy in most patients with pulmonary arterial hypertension (PAH). However, it is unclear to what extend this treatment strategy is adopted in clinical practice and if it is associated with improved long-term survival.MethodsWe analysed data from COMPERA, a large European pulmonary hypertension registry, to assess temporal trends in the use of combination therapy and survival of patients with newly diagnosed PAH between 2010 and 2019. For survival analyses, we look at annualized data and at cumulated data comparing the periods 2010–2014 and 2015–2019.ResultsA total of 2,531 patients were included. The use of early combination therapy (within 3 months after diagnosis) increased from 10.0% in patients diagnosed with PAH in 2010 to 25.0% in patients diagnosed with PAH in 2019. The proportion of patients receiving combination therapy 1 year after diagnosis increased from 27.7% to 46.3%. When comparing the 2010–2014 and 2015–2019 periods, 1-year survival estimates were similar (89.0% [95% CI, 87.2%, 90.9%] and 90.8% [95% CI, 89.3%, 92.4%]), respectively, whereas there was a slight but non-significant improvement in 3-year survival estimates (67.8% [95% CI, 65.0%, 70.8%] and 70.5% [95% CI, 67.8%, 73.4%]), respectively.ConclusionsThe use of combination therapy increased from 2010 to 2019, but most patients still received monotherapy. Survival rates at 1 year after diagnosis did not change over time. Future studies need to determine if the observed trend suggesting improved 3-year survival rates can be confirmed.

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.

scholarly journals Idiopathic Pulmonary Arterial Hypertension Unmasked by Pregnancy

2020 ◽  
Vol 19 (4) ◽  
pp. 240-243
Author(s):  
Adam Maxwell ◽  
◽  
Thomas Holman ◽  
Timea Novak ◽  
◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Post Partum ◽  
Right Heart Failure ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Shortness Of Breath ◽  
Acute Medical Unit ◽  
Pulmonary Angiogram ◽  
Pulmonary Arterial

A 31-year old woman presented to the acute medical unit 9 days post-partum with shortness of breath and peripheral oedema. Initially suspected to have either a pulmonary embolism or post-partum cardiomyopathy, she proceeded to have imaging including a CT Pulmonary angiogram and echocardiogram, which were suggestive of pulmonary hypertension and severe right heart failure. Her history and other investigations did not reveal any obvious cause for this. She was transferred to a specialist centre where she was diagnosed with Idiopathic Pulmonary Arterial Hypertension (IPAH), previously known as primary pulmonary hypertension. Shortness of breath during pregnancy and in the postpartum period is a relatively common acute medical presentation. Whilst IPAH is a rare diagnosis, it carries a high mortality rate, particularly in pregnancy, and requires prompt specialist investigation, diagnosis and management.

scholarly journals Acute vasoreactivity testing in pediatric idiopathic pulmonary arterial hypertension: an international survey on current practice

2019 ◽  
Vol 9 (4) ◽  
pp. 204589401985753
Author(s):  
Lina Caicedo ◽  
Rachel Hopper ◽  
Humberto Garcia Aguilar ◽  
Dunbar Ivy ◽  
Dora Haag ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Clinical Criteria ◽  
First Line Therapy ◽  
Specific Protocol ◽  
The World ◽  
Pulmonary Arterial

The aim of this study was to determine practice patterns and inter-institutional variability in how acute vasoreactivity testing (AVT) is performed and interpreted in pediatrics throughout the world. A survey was offered to physicians affiliated with the Pediatric & Congenital Heart Disease Taskforce of the Pulmonary Vascular Research Institute (PVRI), the Pediatric Pulmonary Hypertension Network (PPHNET), or the Spanish Registry for Pediatric Pulmonary Hypertension (REHIPED), from February to December 2016. The survey requested data about the site-specific protocol for AVT and subsequent management of pediatric patients with idiopathic pulmonary arterial hypertension (IPAH) or heritable PAH (HPAH). Twenty-eight centers from 13 countries answered the survey. AVT is performed in most centers using inhaled nitric oxide (iNO). Sitbon criteria was used in 39% of the centers, Barst criteria in 43%, and other criteria in 18%. First-line therapy for positive AVT responders in functional class (FC) I/II was calcium channel blocker (CCB) in 89%, but only in 68% as monotherapy. Most centers (71%) re-evaluated AVT-positive patients hemodynamics after 6–12 months; 29% of centers re-evaluated based only on clinical criteria. Most centers (64%) considered a good response as remaining in FC I or II, with near normalization of pulmonary arterial pressure and pulmonary vascular resistance, but a stable FC I/II alone was sufficient criteria in 25% of sites. Protocols and diagnostic criteria for AVT, and therapeutic approaches during follow-up, were highly variable across the world. Reported clinical practice is not fully congruent with current guidelines, suggesting the need for additional studies that better define the prognostic value of AVT for pediatric IPAH patients.

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 Genomewide RNA expression profiling in lung identifies distinct signatures in idiopathic pulmonary arterial hypertension and secondary pulmonary hypertension

2010 ◽  
Vol 298 (4) ◽  
pp. H1235-H1248 ◽  
Author(s):  
Revathi Rajkumar ◽  
Kazuhisa Konishi ◽  
Thomas J. Richards ◽  
David C. Ishizawar ◽  
Andrew C. Wiechert ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Estrogen Receptor ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Rna Expression ◽  
Data Set ◽  
Platelet Factor ◽  
Protein Ubiquitination ◽  
Pulmonary Arterial

Idiopathic pulmonary arterial hypertension (PAH) is a life-threatening condition characterized by pulmonary arteriolar remodeling. This investigation aimed to identify genes involved specifically in the pathogenesis of PAH and not other forms of pulmonary hypertension (PH). Using genomewide microarray analysis, we generated the largest data set to date of RNA expression profiles from lung tissue specimens from 1) 18 PAH subjects and 2) 8 subjects with PH secondary to idiopathic pulmonary fibrosis (IPF) and 3) 13 normal subjects. A molecular signature of 4,734 genes discriminated among these three cohorts. We identified significant novel biological changes that were likely to contribute to the pathogenesis of PAH, including regulation of actin-based motility, protein ubiquitination, and cAMP, transforming growth factor-β, MAPK, estrogen receptor, nitric oxide, and PDGF signaling. Bone morphogenic protein receptor type II expression was downregulated, even in subjects without a mutation in this gene. Women with PAH had higher expression levels of estrogen receptor 1 than normal women. Real-time quantitative PCR confirmed differential expression of the following genes in PAH relative to both normal controls and PH secondary to IPF: a disintegrin-like and metalloprotease with thrombospondin type 1 motif 9, cell adhesion molecule with homology to L1CAM, cytochrome b558and β-polypeptide, coagulation factor II receptor-like 3, A-myb myeloblastosis viral oncogene homolog 1, nuclear receptor coactivator 2, purinergic receptor P2Y, platelet factor 4, phospholamban, and tropomodulin 3. This study shows that PAH and PH secondary to IPF are characterized by distinct gene expression signatures, implying distinct pathophysiological mechanisms.

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