scholarly journals Strategy of medical treatment of pulmonary arterial hypertension in the current international recommendations

2016 ◽  
Vol 13 (2) ◽  
pp. 46-64
Author(s):  
T V Martyniuk ◽  
I E Chazova
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Optimal Choice ◽  
New Drugs ◽  
Reverse Remodeling ◽  
Significant Progress ◽  
European Respiratory Society ◽  
Specific Therapy ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology

Significant progress in the treatment of pulmonary arterial hypertension (PAH), in recent years, is associated with the introduction into clinical practice of a number of drugs pathogenetic action, can cause vasodilation and reverse remodeling of pulmonary vessels. Recently PAH-specific therapy was replenished with new drugs. This review is created as a result of the analysis of modern American recommendations CHEST and recommendations of the European society of cardiology and the European respiratory society (ESC/ERS) in order to provide all professionals involved in the maintenance of the PAH, data on the main approaches to the pharmacotherapy and the optimal choice of pharmacological treatment methods.

scholarly journals Dart to the target: an alternative bull’s eye parametric display for European Society of Cardiology / European Respiratory Society goal-orientated risk reduction strategy in pulmonary arterial hypertension

2018 ◽  
Vol 8 (3) ◽  
pp. 204589401878052
Author(s):  
Cihangir Kaymaz ◽  
Ozgur Yasar Akbal ◽  
Aykun Hakgor ◽  
Hacer Ceren Tokgoz ◽  
Seda Tanyeri
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Risk Reduction ◽  
European Society ◽  
European Respiratory Society ◽  
Reduction Strategy ◽  
Risk Reduction Strategy ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology ◽  
Bull's Eye

Despite the significant mortality and mobidity benefits being obtained with the targeted therapies in patients with pulmonary arterial hypertension (PAH), mid- to long-term survival of patients with this disease has remained unsatisfactory. For earlier and reliable risk stratification in PAH and tailoring the dynamic management strategies, various risk assessment models have been developed. Currently available risk reduction strategy recommended by the European Society of Cardiology (ESC)/European Respiratory Society (ERS) 2015 Pulmonary Hypertension Guidelines has been utilized in three recent registries. In this review, we evaluated the risk prediction models and management algorithms in this setting and propose an alternative parametric display, a bull’s eye, dart table scheme for ESC/ERS goal-orientated risk reduction strategy in patients with PAH.

scholarly journals Advanced risk stratification of intermediate risk group in pulmonary arterial hypertension

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402096173
Author(s):  
Athiththan Yogeswaran ◽  
Manuel J. Richter ◽  
Natascha Sommer ◽  
Hossein A. Ghofrani ◽  
Werner Seeger ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pressure Ratio ◽  
Systolic Pulmonary Artery Pressure ◽  
Intermediate Risk ◽  
European Respiratory Society ◽  
Treatment Naïve ◽  
Risk Patients ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology

In 301 treatment-naïve patients with pulmonary arterial hypertension stratified by the European Society of Cardiology/European Respiratory Society risk score, further stratification of intermediate-risk patients based on six-minute walk distance and the tricuspid annular plane systolic excursion/systolic pulmonary artery pressure ratio identified a subset with mortality rates comparable to low-risk patients.

scholarly journals Advanced pulmonary arterial hypertension: mechanical support and lung transplantation

2017 ◽  
Vol 26 (146) ◽  
pp. 170089 ◽  
Author(s):  
Sonja Bartolome ◽  
Marius M. Hoeper ◽  
Walter Klepetko
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Therapeutic Option ◽  
Survival Rates ◽  
Mechanical Support ◽  
European Respiratory Society ◽  
Term Survival ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology

The development of targeted therapies has transformed the outlook for patients with pulmonary arterial hypertension (PAH); however, some patients fail to achieve an adequate clinical response despite receiving maximal treatment. For these patients, lung transplantation remains an important therapeutic option, and recommendations for transplantation are included in the current European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension. Although lung transplantation is not without risk, overall long-term survival rates are good and substantial improvements in quality of life have been reported for lung transplant recipients. In this review, we describe the important considerations prior to, during and after transplantation, including the role of mechanical support, in patients with advanced PAH.

scholarly journals Pregnancy in pulmonary arterial hypertension

2016 ◽  
Vol 25 (142) ◽  
pp. 431-437 ◽  
Author(s):  
Karen M. Olsson ◽  
Richard Channick
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Post Partum ◽  
Early Termination ◽  
European Respiratory Society ◽  
Advanced Therapies ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology ◽  
In Pregnancy

Despite advanced therapies, maternal mortality in women with pulmonary arterial hypertension (PAH) remains high in pregnancy and is especially high during the post-partum period. However, recent data indicates that morbidity and mortality during pregnancy and after birth have improved for PAH patients. The current European Society of Cardiology/European Respiratory Society guidelines recommend that women with PAH should not become pregnant. Therefore, the risks associated with pregnancy must be emphasised and counselling offered to women at the time of PAH diagnosis and to women with PAH who become pregnant. Early termination should be discussed. Women who choose to continue with their pregnancy should be treated at specialised pulmonary hypertension centres with experience in managing PAH during and after pregnancy.

scholarly journals Recent advances in the management of pulmonary arterial hypertension

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 2755 ◽  
Author(s):  
Halley Tsai ◽  
Yon K. Sung ◽  
Vinicio de Jesus Perez
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Soluble Guanylate Cyclase ◽  
Right Heart Failure ◽  
New Drugs ◽  
Triple Combination Therapy ◽  
Treatment Naïve ◽  
Treatment Paradigm ◽  
Pulmonary Arterial

Over the past 20 years, there has been an explosion in the development of therapeutics to treat pulmonary arterial hypertension (PAH), a rare but life-threatening disorder associated with progressive elevation of pulmonary pressures and severe right heart failure. Recently, the field has seen the introduction of riociguat, a soluble guanylate cyclase stimulator, a new endothelin receptor antagonist (macitentan), and oral prostanoids (treprostinil and selexipag). Besides new drugs, there have been significant advances in defining the role of upfront combination therapy in treatment-naïve patients as well as proposed methods to deliver systemic prostanoids by use of implantable pumps. In this review, we will touch upon the most important developments in PAH therapeutics over the last three years and how these have changed the guidelines for the treatment of PAH. These exciting developments herald a new era in the treatment of PAH which will be punctuated by the use of more clinically relevant endpoints in clinical research trials and a novel treatment paradigm that may involve upfront double- or triple-combination therapy. We anticipate that the future will make use of these strategies to test the efficacy of upcoming new drugs that aspire to reduce disease progression and improve survival in patients afflicted with this devastating disease.

scholarly journals Pulmonary Arterial Hypertension Centre – A Model for Rare Disease Management / Centrum pre pľúcnu artériovú hypertenziu – model v manažmente zriedkavých chorôb

2013 ◽  
Vol 60 (Supplementum-VIII) ◽  
pp. 22-25
Author(s):  
E. Goncalvesová
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Disease Management ◽  
Arterial Hypertension ◽  
Rare Disease ◽  
Heart Diseases ◽  
High Volume ◽  
Reference Centre ◽  
Pulmonary Arterial ◽  
Research And Education ◽  
European Society Of Cardiology

Pulmonary arterial hypertension (PAH) is a rare disease with average median survival rate about 3 years from the establishment of the diagnosis, except for PAH associated with congenital heart diseases. Diagnosis and management of PAH concentrate in the dedicated centres (reference centres, centres of expertise). The purpose of a reference centre is to undertake assessment and investigation of all causes of pulmonary hypertension, PAH-specific drug therapy, cooperation with other healthcare specialist, and to undertake research and education. In general, high volume centres achieve best outcomes, because of effective concentration of the specific experience and skills needed for rare disease management. The paper brings brief characteristic of the reference centre for PAH according the guideline of European Society of Cardiology as well as own experience in this field.

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