scholarly journals Infection in pulmonary vascular diseases: Would another consortium really be the way to go?

2019 ◽  
Vol 2019 (1) ◽  
Author(s):  
Ghazwan Butrous ◽  
Alistair Mathie

[first paragraph of article]There has been a clear engagement by the medical community with pulmonary hypertension after the approval of targeted therapies and the introduction of more therapeutic modalities in the last 18 years. The increasing number of scientific sessions and conferences was inevitable. Major initial interest was from the developed countries, which concentrated on prevalent etiologies: primary (later called idiopathic) pulmonary arterial hyposecretion and secondary to connective tissue disorders currently both classified as Class I. Unfortunately, a lesser consideration was given to other causes of pulmonary hypertension such as secondary to left heart failure (Class II) or hypoxic pulmonary disease (Class III). This is presumably due to both the complexity and the multifactorial etiologies of these causes and the lack of availability of targeted therapies. 

2013 ◽  
Vol 305 (2) ◽  
pp. H259-H264 ◽  
Author(s):  
Robert V. MacKenzie Ross ◽  
Mark R. Toshner ◽  
Elaine Soon ◽  
Robert Naeije ◽  
Joanna Pepke-Zaba

This study analyzed the relationship between pulmonary vascular resistance (PVR) and pulmonary arterial compliance ( Ca) in patients with idiopathic pulmonary arterial hypertension (IPAH) and proximal chronic thromboembolic pulmonary hypertension (CTEPH). It has recently been shown that the time constant of the pulmonary circulation (RC time constant), or PVR × Ca, remains unaltered in various forms and severities of pulmonary hypertension, with the exception of left heart failure. We reasoned that increased wave reflection in proximal CTEPH would be another cause of the decreased RC time constant. We conducted a retrospective analysis of invasive pulmonary hemodynamic measurements in IPAH ( n = 78), proximal CTEPH ( n = 91) before (pre) and after (post) pulmonary endarterectomy (PEA), and distal CTEPH ( n = 53). Proximal CTEPH was defined by a postoperative mean pulmonary artery pressure (PAP) of ≤25 mmHg. Outcome measures were the RC time constant, PVR, Ca, and relationship between systolic and mean PAPs. The RC time constant for pre-PEA CTEPH was 0.49 ± 0.11 s compared with post-PEA-CTEPH (0.37 ± 0.11 s, P < 0.0001), IPAH (0.63 ± 0.14 s, P < 0.001), and distal CTEPH (0.55 ± 0.12 s, P < 0.05). A shorter RC time constant was associated with a disproportionate decrease in systolic PAP with respect to mean PAP. We concluded that the pulmonary RC time constant is decreased in proximal CTEPH compared with IPAH, pre- and post-PEA, which may be explained by increased wave reflection but also, importantly, by persistent structural changes after the removal of proximal obstructions. A reduced RC time constant in CTEPH is in accord with a wider pulse pressure and hence greater right ventricular work for a given mean PAP.


2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Sudhiranjan Gupta ◽  
Li Li

MicroRNAs (miRNAs) have emerged as a new class of posttranscriptional regulators of many cardiac and vascular diseases. They are a class of small, noncoding RNAs that contributes crucial roles typically through binding of the 3′-untranslated region of mRNA. A single miRNA may influence several signaling pathways associated with cardiac remodeling by targeting multiple genes. Pulmonary hypertension (PH) is a rare disorder characterized by progressive obliteration of pulmonary (micro) vasculature that results in elevated vascular resistance, leading to right ventricular hypertrophy (RVH) and RV failure. The pathology of PH involves vascular cell remodeling including pulmonary arterial endothelial cell (PAEC) dysfunction and pulmonary arterial smooth muscle cell (PASMC) proliferation. There is no cure for this disease. Thus, novel intervention pathways that govern PH induced RVH may result in new treatment modalities. Current therapies are limited to reverse the vascular remodeling. Recent studies have demonstrated the roles of various miRNAs in the pathogenesis of PH and pulmonary disorders. This review provides an overview of recent discoveries on the role of miRNAs in the pathogenesis of PH and discusses the potential for miRNAs as therapeutic targets and biomarkers of PH at clinical setting.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Aya Miura ◽  
Kazufumi Nakamura ◽  
Kengo F Kusano ◽  
Hiromi Matsubara ◽  
Daiji Miura ◽  
...  

Background and Objectives: Pulmonary vascular diseases (pulmonary arterial hypertension: PAH, pulmonary veno-occlusive disease: PVOD and pulmonary capillary hemangiomatosis: PCH) are well-known diseases that cause pulmonary hypertension (PH). However, the differences of structural characteristics of pulmonary microvasculature in these diseases have not been elucidated enough. We examined the morphological differences among these diseases by methacrylate resin (Mercox) perfusion using scanning electron microscope. Methods and Results: Ten lung samples were 8 patients with PH and 2 control subjects without PH. These were obtained at lung transplantations or autopsies. Microvascular corrosion replicas were made by Mercox perfusion from the pulmonary artery. Pathological diagnosis revealed 5 PAH, 2 PVOD and 1 PCH in patients with PH. In PAH samples, small arteries or arterioles showed twisted and tortuous courses, but such vessels were not observed in samples from control, PVOD and PCH. Binary branching was observed in control, but not in PVOD and PCH. In PVOD and PCH, several branching was commonly observed. Moreover, the loss of capillary network and the disruption of arterioles were more severe in samples from PVOD and PCH patients than in samples from PAH patients. Conclusion: Pulmonary microvascular morphology is seriously different among the type of underlying diseases in PH patients.


ESC CardioMed ◽  
2018 ◽  
pp. 2484-2487
Author(s):  
Jose Leonidas Alves ◽  
Susana Hoette ◽  
Rogério Souza

The epidemiology of pulmonary hypertension has been better studied in the last decade. Registries coming from all parts of the world allowed not only the recognition of a change in pulmonary hypertension demographics in developed countries but also the existence of regional peculiarities in less developed regions that need to be accounted for in the development of appropriate health policies. Although left-sided heart diseases and lung diseases are believed to be the most prevalent causes of pulmonary hypertension, most of the available data are related to causes of pulmonary arterial hypertension. In this particular group, patients are now diagnosed at an older age, as compared with the data from the first national registries, from about 20 years ago. The reasons for such changes are not clear and might be related to factors such as disease awareness, diagnostic approach, treatment availability, and health system accessibility rather than to a change in the disease itself. This chapter summarizes the available data regarding the current epidemiology of pulmonary hypertension.


2011 ◽  
Vol 10 (1) ◽  
pp. 41-45
Author(s):  
Hunter C. Champion

Pulmonary hypertension (PH) commonly results as a complication of left heart failure (systolic or nonsystolic dysfunction). It is important to note that there is a distinct difference between PH as a whole and pulmonary arterial hypertension (PAH), which have the same criteria with regard to mean pulmonary artery pressure (mPAP) as PAH with the critical difference of the pulmonary capillary wedge pressure (PCWP) measurement. Table 1 shows the most common causes of RV dysfunction. The issue of management of right ventricular (RV) failure has recently become more important with increased awareness of RV failure symptoms in the setting of PH. Moreover, with growing consideration for surgical left ventricular support (left ventricular assist device, LVAD) and the need for RV functional competence, the need to better understand the role of RV function is becoming more paramount.


F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 888 ◽  
Author(s):  
David F Condon ◽  
Nils P Nickel ◽  
Ryan Anderson ◽  
Shireen Mirza ◽  
Vinicio A de Jesus Perez

In February 2018, the 6th World Symposium on Pulmonary Hypertension (WSPH) brought together experts from various disciplines to review the most relevant clinical and scientific advances in the field of PH over the last 5 years. Based on careful review and discussions by members of the different task forces, major revisions were made on the hemodynamic definition for various forms of PH and new genes were added to the list of genetic markers associated with pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease. In addition, the use of risk stratification tools was encouraged as a strategy to reduce one-year mortality risk in PAH patients through early implementation of PAH therapies. While members of the medical community are still debating some of the proposed changes, the new WSPH guidelines advocate early diagnosis and initiation of combination therapy to reduce mortality and improve quality of life in patients with PH.


2020 ◽  
Vol 71 (2) ◽  
pp. 413-417
Author(s):  
Alina Costina Luca ◽  
Lucian Eva ◽  
Letitia Doina Duceac ◽  
Geta Mitrea ◽  
Constantin Marcu ◽  
...  

Pulmonary arterial hypertension is a disorder of high mortality being described by a progressive increase in pulmonary vascular strength leading to right ventricular damage and death. Endotelin-1 (ET-1) has an important pathogenic fuction in pulmonary hypertension. Bosentan is an oral ET-1 receptor antagonist that proved to be efficient at exercise tolerance in patients with pulmonary hypertension in functional class III and IV. The early trial registered for functional class II patients and evaluated hemodynamic at 6 month. Considerable side effects of bosentan comprise anemia, edema, and transaminase raise. Further studies were developed to determine optimum treatment strategy by eliminating adverse effects of bosentan. The major aim of this work was to prepare bosentan loaded nanoparticles by solvent evaporation technique for a sustained release of the drug by using ethyl cellulose as polymer. Drug and polymer were dissolved in ethanol at different drug-polymer ratios i.e. 1:2 and 1:3. Among the two formulations, 1:3 formulations were appreciated as the best formulation with better drug content (97.5%) and incorporation efficiency (95.5%).


Author(s):  
Sara Thorne ◽  
Sarah Bowater

Pulmonary arterial hypertension (PAH) is a common complication in ACHD patients, with a prevalence of around 10% in developed countries. It is most commonly due to large, uncorrected left-to-right shunts, although a permissive genotype might explain its development in other, seemingly lower-risk, patients. Histologically, PAH in ACHD is the same as PAH due to other aetiologies, such as idiopathic and connective tissue disease.


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