scholarly journals Pulmonary hypertension

2016 ◽  
Vol 25 (139) ◽  
pp. 4-11 ◽  
Author(s):  
Anton Vonk Noordegraaf ◽  
Joanne A. Groeneveldt ◽  
Harm Jan Bogaard
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Review Article ◽  
Pulmonary Vasculature ◽  
Distant Future ◽  
European Guidelines ◽  
Enormous Amount ◽  
Pulmonary Arterial ◽  
The Right

In 2015, more than 800 papers were published in the field of pulmonary hypertension. A Clinical Year in Review article cannot possibly incorporate all this work and needs to be selective. The recently published European guidelines for the diagnosis and treatment of pulmonary hypertension contain an inclusive summary of all published clinical studies conducted until very recently. Here, we provide an overview of papers published after the finalisation of the guideline. In addition, we summarise recent advances in pulmonary vasculature science. The selection we made from the enormous amount of published work undoubtedly reflects our personal views and may not include all papers with a significant impact in the near or more distant future. The focus of this paper is on the diagnosis of pulmonary arterial hypertension, understanding the success of combination therapy on the right ventricle and scientific breakthroughs.

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 Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension

2021 ◽  
Vol 30 (162) ◽  
pp. 210161
Author(s):  
Frédéric Perros ◽  
Marc Humbert ◽  
Peter Dorfmüller
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Rare Condition ◽  
Progressive Increase ◽  
Pulmonary Vasculature ◽  
Ventricular Failure ◽  
Myocardial Remodelling ◽  
Pulmonary Arterial ◽  
The Right

Pulmonary arterial hypertension (PAH) is a rare condition that is characterised by a progressive increase of pulmonary vascular resistances that leads to right ventricular failure and death, if untreated. The underlying narrowing of the pulmonary vasculature relies on several independent and interdependent biological pathways, such as genetic predisposition and epigenetic changes, imbalance of vasodilating and vasoconstrictive mediators, as well as dysimmunity and inflammation that will trigger endothelial dysfunction, smooth muscle cell proliferation, fibroblast activation and collagen deposition. Progressive constriction of the pulmonary vasculature, in turn, initiates and sustains hypertrophic and maladaptive myocardial remodelling of the right ventricle. In this review, we focus on the role of inflammation and dysimmunity in PAH which is generally accepted today, although existing PAH-specific medical therapies still lack targeted immune-modulating approaches.

scholarly journals Chronic thromboembolic pulmonary hypertension: a distinct disease entity

2015 ◽  
Vol 24 (136) ◽  
pp. 246-252 ◽  
Author(s):  
Irene Lang
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Pulmonary Vasculature ◽  
Distinct Disease ◽  
Thromboembolic Pulmonary Hypertension ◽  
Perfusion Scan ◽  
Pulmonary Arterial

Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct subtype of pulmonary hypertension (PH). One disease hypothesis is that CTEPH results from the non-resolution of venous thromboembolism. CTEPH is characterised by the presence of obstructive fibrotic thromboembolic material in the major pulmonary vessels, with concomitant microvascular arteriopathy, resulting in progressive PH. The clinical presentation of CTEPH is similar to pulmonary arterial hypertension with nonspecific symptoms, but it is distinguished from pulmonary arterial hypertension by the presence of mismatched segmental defects on the ventilation/perfusion scan. The exact prevalence and incidence of CTEPH are unknown, but are thought to have been underestimated in the past. CTEPH is unique among the subgroups of PH in that it is potentially curable with pulmonary endarterectomy, a surgical intervention intended to remove the occlusive material from the pulmonary vasculature. However, in some patients the obstructions are technically inaccessible or the risk/benefit ratios are unfavourable, making the condition inoperable. It is thought that the involvement of the smaller, more distal vessels is a target for medical treatment. Untreated, CTEPH may result in right heart failure and death. The pathophysiological mechanisms which cause CTEPH are complex and have not yet been fully elucidated.

scholarly journals Echocardiography in Pulmonary Arterial Hypertension: Is It Time to Reconsider Its Prognostic Utility?

2021 ◽  
Vol 10 (13) ◽  
pp. 2826
Author(s):  
Ioannis T. Farmakis ◽  
Eftychia Demerouti ◽  
Panagiotis Karyofyllis ◽  
George Karatasakis ◽  
Maria Stratinaki ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Heart Failure ◽  
Pulmonary Vasculature ◽  
Right Heart ◽  
Prognostic Utility ◽  
Pulmonary Arterial ◽  
Prognostic Implications ◽  
The Right

Pulmonary arterial hypertension (PAH) is characterized by an insult in the pulmonary vasculature, with subsequent right ventricular (RV) adaptation to the increased afterload that ultimately leads to RV failure. The awareness of the importance of RV function in PAH has increased considerably because right heart failure is the predominant cause of death in PAH patients. Given its wide availability and reduced cost, echocardiography is of paramount importance in the evaluation of the right heart in PAH. Several echocardiographic parameters have been shown to have prognostic implications in PAH; however, the role of echocardiography in the risk assessment of the PAH patient is limited under the current guidelines. This review discusses the echocardiographic evaluation of the RV in PAH and during therapy, and its prognostic implications, as well as the potential significant role of repeated echocardiographic assessment in the follow-up of patients with PAH.

scholarly journals Consensus or Controversy: Do Recent Advances Shift the Debate for the Use of Echocardiography Versus Cardiac Magnetic Resonance Imaging of the Right Ventricle in Pulmonary Arterial Hypertension?

2015 ◽  
Vol 14 (1) ◽  
pp. 28-36
Author(s):  
Amresh Raina ◽  
Benjamin Freed
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Right Ventricle ◽  
Arterial Hypertension ◽  
Surrogate Endpoint ◽  
Pulmonary Vasculature ◽  
Technological Advances ◽  
The Status ◽  
Pulmonary Arterial ◽  
The Right ◽  
Insight Into

Pulmonary arterial hypertension (PAH) is a progressive, often lethal condition originating in the pulmonary arteriolar tree. It is typically manifested in stereotypical changes in the right ventricle (RV). RV dysfunction is an important mediator of patient symptoms in PAH, and RV failure is the most common cause of mortality in PAH patients. Because of the physiologic importance of the RV, RV imaging is critical in the initial diagnostic evaluation and serial assessment of PAH patients, and can provide indirect insight into the status of the disease at the level of the pulmonary vasculature. This article will focus on whether technological advances in imaging have shifted the debate toward which modality is optimal both for routine clinical practice and for a possible surrogate endpoint in PAH clinical trials.

scholarly journals The pathophysiological role of novel pulmonary arterial hypertension gene SOX17

2021 ◽  
pp. 2004172
Author(s):  
Yukyee Wu ◽  
John Wharton ◽  
Rachel Walters ◽  
Eleni Vasilaki ◽  
Jurjan Aman ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Dominant Mode ◽  
Pulmonary Vasculature ◽  
Incomplete Penetrance ◽  
Pathophysiological Role ◽  
Sequencing Studies ◽  
Pulmonary Arterial ◽  
The Right

Pulmonary arterial hypertension (PAH) is a progressive disease predominantly targeting pre-capillary blood vessels. Adverse structural re-modelling and increased pulmonary vascular resistance result in cardiac hypertrophy and ultimately failure of the right ventricle. Recent whole genome and exome sequencing studies have identified SOX17 as a novel risk gene in PAH, with a dominant mode of inheritance and incomplete penetrance. Rare deleterious variants in the gene and more common variants in upstream enhancer sites have both been associated with the disease and a deficiency of SOX17 expression may predispose to PAH. This review aims to consolidate the evidence linking genetic variants in SOX17 to PAH and explores the numerous targets and effects of the transcription factor, focussing on the pulmonary vasculature and the pathobiology of PAH.

scholarly journals Features of clinical, functional and hemodynamics profile, medical treatment and prognosis evaluation in patients with inoperable chronic thromboembolic pulmonary hypertension and idiopathic pulmonary arterial hypertension according to the Russian registry

2019 ◽  
Vol 91 (9) ◽  
pp. 77-87 ◽  
Author(s):  
I E Chazova ◽  
Z S Valieva ◽  
S N Nakonechnikov ◽  
I N Taran ◽  
T V Martynyuk
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Pulmonary Arterial Pressure ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Mean Pulmonary Arterial Pressure ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
The Right

Aim: to perform the complex analysis of clinical, functional, hemodynamics profile in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) compared to the idiopathic pulmonary arterial hypertension (IPAH) group at the time of diagnosis verification according to the Russian registry, and to evaluate the features of medical therapy and it’s influence on prognosis in these patients. Materials and methods. In the retrospective study 193 patients with IPAH and 130 patients with inoperable CTEPH older than 18 years were included. All included patients were the participants of Russian registry (www.medibase.pro) in 15 Russian expert centers during the period from 01.01.2012 to 31.12.2018 year. The diagnosis was verified according to the algorithm, reflected in the Russian guidelines on diagnosis and treatment of PH and CTEPH (2016 year). The comparison analysis of clinical, functional, hemodynamics parameters in patients with IPAH and inoperable CTEPH was made. Results. The status of 193 patients with IPAH (32 male and 162 female) and 130 patients with inoperable CTEPH - (40 male and 90 female) was analyzed during the study. The CTEPH patients were older compared to the IPAH patients: 52.2 [41.1; 60.6] and 36.5 [26.8; 36.5] years, respectively. The median period since symptom occurrence till CTEPH verification was 1.08 [0.2; 3.1] years, in IPAH patients - 2.01 [0.6; 4.2] years. More than 80% of inoperable CTEPH patients had III and IV functional class (FC) according to the World Health Organization classification at the time of diagnosis verification versus 61% of IPAH patients. According to echocardiography the level of mean pulmonary arterial pressure was comparable in two groups of PH patients. However in inoperable CTEPH patients the right atrial area was larger. The significantly higher value of mean pulmonary arterial pressure and lower value oxygen saturation of arterial blood according to the right heart catheterization were revealed. The 5-year survival in CTEPH patients, receiving initial dual combination therapy in 75% cases (in 40% - combination of riociguat and iloprost ) was 93% versus 86.5% in patients with IPAH. Conclusion: It was revealed, that inoperable CTEPH patients were significantly older with severe functional and hemodynamics status at the time of diagnosis verification, although with higher level of 5-year survival compared to the IPAH patients according to the Russian registry.

scholarly journals Pulmonary Arterial Hypertension and Therapeutic Interventions

2019 ◽  
Vol 28 (02) ◽  
pp. 080-092 ◽  
Author(s):  
Nader B. Ishak Gabra ◽  
Omar Mahmoud ◽  
Oki Ishikawa ◽  
Varun Shah ◽  
Erica Altshul ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Review Article ◽  
Therapeutic Interventions ◽  
Management Options ◽  
Future Directions ◽  
Uncommon Disease ◽  
Disease Entities ◽  
Pulmonary Arterial

AbstractPulmonary hypertension is an uncommon disease that carries a significant morbidity and mortality. Pulmonary arterial hypertension is a subtype of pulmonary hypertension that describes a group of disease entities that lead to an elevation in precapillary pulmonary artery pressure. Despite advances in the diagnosis and treatment of pulmonary arterial hypertension, it remains a difficult disease to recognize and manage. In this review article, we will discuss the definition and diagnosis of pulmonary arterial hypertension. Additionally, we will discuss the ever-expanding management options, their mechanisms and strategies, including combination therapy and the most recent advances and future directions.

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.

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