Present and Future Treatment Strategies for Pulmonary Arterial Hypertension

2006 ◽  
Vol 5 (4) ◽  
pp. 271-282 ◽  
Author(s):  
Laura B Kane ◽  
Elizabeth S Klings
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Treatment Strategies ◽  
Future Treatment ◽  
Pulmonary Arterial

Pulmonary vasculature

2017 ◽  
Author(s):  
Sophie Vergnaud ◽  
David Dobarro ◽  
John Wort
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Treatment Strategies ◽  
Symptomatic Improvement ◽  
Pulmonary Vasculature ◽  
History Of ◽  
Diffuse Cutaneous Systemic Sclerosis ◽  
Pulmonary Arterial ◽  
Phosphodiesterase 5 ◽  
Haemodynamic Improvement

A 16-year-old girl with a diagnosis of diffuse cutaneous systemic sclerosis is referred to a specialist pulmonary hypertension centre with a history of progressive breathlessness, reduced exercise tolerance, and raised pulmonary pressures on transthoracic echocardiogram. She is found to have pulmonary arterial hypertension on right cardiac catheterization and is started on sildenafil, a phosphodiesterase-5 inhibitor, which stabilizes her condition. An endothelin receptor antagonist is added, which provides some initial symptomatic improvement. She continues to deteriorate over a period of 5 years, ultimately requiring intravenous prostanoids, the only treatment to provide a real symptomatic and haemodynamic improvement. This chapter explores the physiology and pathophysiology of pulmonary arterial hypertension, its classification, the means of investigation and diagnosis, who to refer to specialist centres, and the concepts behind current and future treatment strategies.

Identification of treatment goals in paediatric pulmonary arterial hypertension

2014 ◽  
Vol 44 (6) ◽  
pp. 1616-1626 ◽  
Author(s):  
Mark-Jan Ploegstra ◽  
Johannes M. Douwes ◽  
Marcus T.R. Roofthooft ◽  
Willemijn M.H. Zijlstra ◽  
Hans L. Hillege ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Treatment Strategies ◽  
World Health ◽  
Treatment Goals ◽  
Predictive Values ◽  
Free Survival ◽  
Pulmonary Arterial ◽  
Induced Changes

To be able to design goal-oriented treatment strategies in paediatric pulmonary arterial hypertension (PAH), we aimed to identify treatment goals by investigating the prognostic value of treatment-induced changes in noninvasive predictors of transplant-free survival.66 consecutive, treatment-naïve paediatric PAH patients in the Dutch National Network for Paediatric Pulmonary Hypertension who started taking PAH-targeted drugs between January 2000 and April 2013 underwent prospective, standardised follow-up. Clinical, biochemical and echocardiographic measures were longitudinally collected at treatment initiation and follow-up, and their respective predictive values for transplant-free survival were assessed. Furthermore, the predictive values of treatment-induced changes were assessed.From the identified set of baseline predictors, the variables World Health Organization functional class (WHO-FC), N-terminal pro-brain natriuretic peptide (NT-proBNP) and tricuspid annular plane systolic excursion (TAPSE) were identified as follow-up predictors in which treatment-induced changes were associated with survival. Patients in whom these variables improved after treatment showed better survival (p<0.002).Therefore, WHO-FC, NT-proBNP and TAPSE are not only predictors of transplant-free survival in paediatric PAH but can also be used as treatment goals, as treatment-induced improvements in these variables are associated with improved survival. The identification of these variables allows for the introduction of goal-oriented treatment strategies in paediatric PAH.

scholarly journals Bioactivities and mechanisms of natural medicines in the management of pulmonary arterial hypertension

2022 ◽  
Vol 17 (1) ◽  
Author(s):  
Zhijie Yu ◽  
Jun Xiao ◽  
Xiao Chen ◽  
Yi Ruan ◽  
Yang Chen ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Animal Models ◽  
Arterial Hypertension ◽  
Clinical Symptoms ◽  
Treatment Strategies ◽  
Terminal Phase ◽  
Pharmacokinetic Studies ◽  
Natural Medicine ◽  
Pulmonary Arterial ◽  
Natural Medicines

AbstractPulmonary arterial hypertension (PAH) is a progressive and rare disease without obvious clinical symptoms that shares characteristics with pulmonary vascular remodeling. Right heart failure in the terminal phase of PAH seriously threatens the lives of patients. This review attempts to comprehensively outline the current state of knowledge on PAH its pathology, pathogenesis, natural medicines therapy, mechanisms and clinical studies to provide potential treatment strategies. Although PAH and pulmonary hypertension have similar pathological features, PAH exhibits significantly elevated pulmonary vascular resistance caused by vascular stenosis and occlusion. Currently, the pathogenesis of PAH is thought to involve multiple factors, primarily including genetic/epigenetic factors, vascular cellular dysregulation, metabolic dysfunction, even inflammation and immunization. Yet many issues regarding PAH need to be clarified, such as the “oestrogen paradox”. About 25 kinds monomers derived from natural medicine have been verified to protect against to PAH via modulating BMPR2/Smad, HIF-1α, PI3K/Akt/mTOR and eNOS/NO/cGMP signalling pathways. Yet limited and single PAH animal models may not corroborate the efficacy of natural medicines, and those natural compounds how to regulate crucial genes, proteins and even microRNA and lncRNA still need to put great attention. Additionally, pharmacokinetic studies and safety evaluation of natural medicines for the treatment of PAH should be undertaken in future studies. Meanwhile, methods for validating the efficacy of natural drugs in multiple PAH animal models and precise clinical design are also urgently needed to promote advances in PAH. Graphical Abstract

scholarly journals Treatment and Palliation of Symptoms in Patients With Advanced Pulmonary Arterial Hypertension

2018 ◽  
Vol 17 (1) ◽  
pp. 20-24
Author(s):  
Kasey L. Malotte ◽  
Aaron Weinberg ◽  
Victor F. Tapson
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Complex Disease ◽  
Treatment Strategies ◽  
Symptom Burden ◽  
Supportive Treatment ◽  
Patient Case ◽  
Pulmonary Arterial ◽  
Extended Survival

Pulmonary arterial hypertension (PAH) is a complex disease in which the illness as well as the associated treatment can be particularly burdensome. Previously considered to be uniformly fatal, PAH treatment and management has improved with an extended survival. However, this can come with symptom burden. Burden can be associated with the disease itself in addition to the treatment aimed at ameliorating these symptoms. This article explores both the use of PAH-directed therapy as well as traditionally supportive treatment strategies for palliation of symptoms in those with PAH. As highlighted in the patient case described throughout this issue, patients are constantly weighing the benefits and burdens of treatment during each step of their journey.

scholarly journals My life with pulmonary arterial hypertension: a patient perspective

2019 ◽  
Vol 21 (Supplement_K) ◽  
pp. K54-K59
Author(s):  
Pisana Ferrari ◽  
Hall Skåra
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Healthcare Professionals ◽  
Treatment Guidelines ◽  
Guideline Development ◽  
Treatment Strategies ◽  
Patient Treatment ◽  
Dietary Recommendations ◽  
Patient Representatives ◽  
Pulmonary Arterial

Abstract In recent years, there has been an increase in therapeutic options and treatment strategies for pulmonary arterial hypertension (PAH). However, patients still report delays in receiving a diagnosis, which is a significant burden associated with the disease, and which shows a general lack of disease awareness. This review has been written by two PAH patients to describe the patient experience and explore the ways in which patients are increasingly being given a voice in developing approaches to treatment. As patients with PAH are living longer, it is important that they work with healthcare professionals to develop treatment strategies that improve and maintain quality of life. Healthcare professionals should consider a holistic approach to disease management, including dietary recommendations, individually adapted exercise, and options for counselling where available, alongside therapeutic treatments. The experiences of patients with PAH are important not just for individual patient treatment but should also be considered in clinical trial design and guideline development. Patient representatives and patient associations can play an influential role in improving the treatment and management of PAH. In this review, we use our experiences as patient representatives to describe the current situation of patients with PAH from first experiencing symptoms to receiving treatment, using two patient cases as examples. We also discuss the role of patient advocacy in improving PAH care and future roles for patient associations and patient representatives in the design of clinical trials and development of new treatment guidelines.

The hallmarks of severe pulmonary arterial hypertension: the cancer hypothesis—ten years later

2020 ◽  
Vol 318 (6) ◽  
pp. L1115-L1130 ◽  
Author(s):  
Carlyne D. Cool ◽  
Wolfgang M. Kuebler ◽  
Harm Jan Bogaard ◽  
Edda Spiekerkoetter ◽  
Mark R. Nicolls ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Cell Injury ◽  
Vascular Endothelial Cell ◽  
Treatment Strategies ◽  
Vascular Wall ◽  
Vascular Endothelial ◽  
Proliferating Cells ◽  
Bone Marrow Derived Cells ◽  
Pulmonary Arterial

Severe forms of pulmonary arterial hypertension (PAH) are most frequently the consequence of a lumen-obliterating angiopathy. One pathobiological model is that the initial pulmonary vascular endothelial cell injury and apoptosis is followed by the evolution of phenotypically altered, apoptosis-resistant, proliferating cells and an inflammatory vascular immune response. Although there may be a vasoconstrictive disease component, the increased pulmonary vascular shear stress in established PAH is caused largely by the vascular wall pathology. In this review, we revisit the “quasi-malignancy concept” of severe PAH and examine to what extent the hallmarks of PAH can be compared with the hallmarks of cancer. The cancer model of severe PAH, based on the growth of abnormal vascular and bone marrow-derived cells, may enable the emergence of novel cell-based PAH treatment strategies.

scholarly journals The Growing Role of Echocardiography in Pulmonary Arterial Hypertension Risk Stratification: The Missing Piece

2021 ◽  
Vol 10 (4) ◽  
pp. 619
Author(s):  
Cristiano Miotti ◽  
Silvia Papa ◽  
Giovanna Manzi ◽  
Gianmarco Scoccia ◽  
Federico Luongo ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Right Ventricle ◽  
Arterial Hypertension ◽  
Treatment Strategies ◽  
Right Heart Failure ◽  
Risk Scores ◽  
Reverse Remodeling ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
Current Risk

Pulmonary arterial hypertension (PAH) is a rare, progressive disease with a poor prognosis. The pathophysiologic model is mainly characterized by an afterload mismatch in which an increased right ventricle afterload, driven by increased pulmonary vascular resistance (PVR), leads to right heart failure. International guidelines recommend optimization of treatment based on regular risk assessments to achieve or maintain a low-risk status. Current risk scores are based on a multi-modality approach, including demographic, clinical, functional, exercise, laboratory, and hemodynamic parameters, which lack significant echocardiographic parameters. The originality of echocardiography relies on the opportunity to assess in a non-invasive way a physiologically meaningful combination of easy to measure variables tightly related to right ventricle adaptation/maladaptation to increased afterload, the main determinant of a patient’s prognosis. Echo-derived morphological and functional parameters have been investigated in PAH, proving to have prognostic relevance. Different therapeutic strategies proved to have different effects in reducing PVR. An upfront combination of drugs, including a parenteral prostacyclin, has shown to be associated with right heart reverse remodeling in a greater proportion of patients than other treatment strategies as a function of PVR reduction. Adding echocardiographic data to current risk scores would allow better identification of right ventricle (RV) adaptation in PAH patients’ follow-up. This additional information would allow better stratification of the patient, leading to optimized and personalized therapeutic management.

scholarly journals Burden of pulmonary arterial hypertension in England: retrospective HES database analysis

2021 ◽  
Vol 15 ◽  
pp. 175346662199504
Author(s):  
Fernando Exposto ◽  
Ruben Hermans ◽  
Åsa Nordgren ◽  
Luke Taylor ◽  
Sanam Sikander Rehman ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Economic Burden ◽  
Treatment Strategies ◽  
The United States ◽  
Resource Utilisation ◽  
Healthcare Resource Utilisation ◽  
Outpatient Visits ◽  
Specialist Centre ◽  
Pulmonary Arterial

Background: The clinical and economic burden of pulmonary arterial hypertension (PAH) is poorly understood outside the United States. This retrospective database study describes the characteristics of patients with PAH in England, including their healthcare resource utilisation (HCRU) and associated costs. Methods: Data from 1 April 2012 to 31 March 2018 were obtained from the National Health Service (NHS) Digital Hospital Episode Statistics database, which provides full coverage of patient events occurring in NHS England hospitals. An adult patient cohort was defined using an algorithm incorporating pulmonary hypertension (PH) diagnosis codes, PAH-associated procedures, PH specialist centre visits and PAH-specific medications. HCRU included inpatient admissions, outpatient visits and Accident and Emergency (A&E) attendances. Associated costs, calculated using national tariffs inflation-adjusted to 2017, did not include PAH-specific drugs on the High Cost Drugs list. Results: The analysis cohort included 2527 patients (68.4% female; 63.6% aged ⩾50 years). Mean annual HCRU rates ranged from 2.9 to 3.2 for admissions (21–25% of patients had ⩾5 admissions), 9.4–10.3 for outpatient visits and 0.8–0.9 for A&E attendances. Costs from 2013 to 2017 totalled £43.2M (£33.9M admissions, £8.3M outpatient visits and £0.9M A&E attendances). From 2013 to 2017, mean cost per patient decreased 13% (from £4400 to £3833) for admissions and 13% (from £1031 to £896) for outpatient visits, but increased 52% (from £81 to £123) for A&E attendances. Conclusion: PAH incurs a heavy economic burden on a per-patient basis, highlighting the need for improved treatment strategies able to reduce disease progression and hospitalisations. The reviews of this paper are available via the supplemental material section.

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