scholarly journals New Treatment Strategies for Pulmonary Arterial Hypertension

2013 ◽  
Vol 62 (12) ◽  
pp. 1101-1102 ◽  
Author(s):  
Nazzareno Galiè ◽  
Alessandra Manes
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Treatment Strategies ◽  
Pulmonary Arterial ◽  
New Treatment

Recent Updates in Both the Diagnosis and Treatment Options that Impact the Clinical Treatment of Pulmonary Arterial Hypertension

2010 ◽  
Vol 2 ◽  
pp. CMT.S4192
Author(s):  
Charles D. Burger
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Treatment Plan ◽  
Treatment Options ◽  
Pharmaceutical Formulations ◽  
World Health ◽  
New Agents ◽  
Pulmonary Arterial ◽  
New Treatment ◽  
Health Organization

The evaluation and management of pulmonary arterial hypertension (PAH) is a rapidly evolving area of subspecialty medicine requiring regular clinical updates. Most notably are changes in the World Health Organization diagnostic scheme whereby the clinician categorizes the correct type of pulmonary hypertension in order direct the most specific evaluation and treatment plan. In addition, there have been several changes in both the FDA-approved pharmaceutical formulations and new agents for the treatment of PAH. This review will provide an update in these areas and more importantly, guidance to the clinician on the most appropriate utilization of these new treatment options.

scholarly journals A new treatment for severe pulmonary arterial hypertension based on an old idea: inhibition of 5-lipoxygenase

2020 ◽  
Vol 10 (1) ◽  
pp. 204589401988263
Author(s):  
Norbert F. Voelkel ◽  
Marc Peters-Golden
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lipoxygenase Inhibitors ◽  
Mediators Of Inflammation ◽  
Background Data ◽  
Receptor Blockers ◽  
Pulmonary Arterial ◽  
Leukotriene Receptor ◽  
New Treatment ◽  
Severe Pulmonary Arterial Hypertension

It has been generally accepted that severe forms of pulmonary arterial hypertension are associated with inflammation. Plasma levels in patients with severe pulmonary arterial hypertension show elevated levels of interleukins and mediators of inflammation and histologically the diseased small pulmonary arterioles show infiltrates of inflammatory and immune cells. Here, we review the literature that connects pulmonary hypertension with the arachidonic acid/5-lipoxygenase-derived leukotriens. This mostly preclinical background data together with the availability of 5-lipoxygenase inhibitors and leukotriene receptor blockers provide the rationale for testing the hypothesis that 5-lipoxygenase products contribute to the pathobiology of severe pulmonary arterial hypertension in a subgroup of patients.

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.

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