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Cellular Pathogenesis of Pulmonary Arterial Hypertension
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Cellular Pathogenesis of Pulmonary Arterial Hypertension

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cellular Pathology".

Deadline for manuscript submissions: closed (15 July 2021) | Viewed by 46142

Special Issue Editor

Dr. Rozenn Quarck

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Guest Editor
Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Dept Chronic Diseases & Metabolism (CHROMETA), University of Leuven—KU Leuven, Leuven, Belgium
Interests: pulmonary arterial hypertension; chronic thromboembolic pulmonary hypertension; angiogenesis; inflammation
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Pulmonary arterial hypertension is a rare and devastating condition, characterized by the remodeling and the obstruction of pulmonary microvessels and resulting in increased pulmonary vascular resistance, elevated pulmonary arterial pressures, and right ventricular failure. Despite the development of various drugs during the past 3 decades, the disease cannot be cured and the prognosis remains unacceptable, with currently available drugs displaying vasodilatory effects but failing to target the pulmonary vascular remodeling. Pulmonary arterial hypertension can be either idiopathic, heritable or associated with other conditions such as connective tissue diseases, congenital heart diseases, drug/toxin intake, portal hypertension, schistosomiasis, and human immunodeficiency virus. When PAH occurs in a hereditary context, germline mutations in various genes have been identified. The pathophysiological process of PAH is an intricate process that involves vasoconstriction, vascular remodeling, endothelial dysfunction, inflammation, and thrombosis.

This Special Issue aims to provide comprehensible knowledge regarding recently identified mechanisms of the cellular pathogenesis of pulmonary arterial hypertension, which could open future and innovative treatment options and strategies.

Consequently, we warmly invite the community to submit original articles or reviews covering the abovementioned field. Please make sure that the paper matches the scope of our journal. https://www.mdpi.com/journal/cells/about

We look forward to your contributions.

Dr. Rozenn Quarck
Guest Editor

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Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cells is an international peer-reviewed open access semimonthly journal published by MDPI.

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Keywords

  • pulmonary arterial hypertension
  • inflammation
  • angiogenesis
  • endothelium
  • extra cellular matrix
  • vascular remodeling

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Published Papers (10 papers)

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Research

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19 pages, 6499 KiB  
Article
Development of a 3-Dimensional Model to Study Right Heart Dysfunction in Pulmonary Arterial Hypertension: First Observations
by Aida Llucià-Valldeperas, Rowan Smal, Fjodor T. Bekedam, Margaux Cé, Xiaoke Pan, Xue D. Manz, Paul J. M. Wijnker, Anton Vonk-Noordegraaf, Harm J. Bogaard, Marie-Jose Goumans and Frances S. de Man
Cells 2021, 10(12), 3595; https://doi.org/10.3390/cells10123595 - 20 Dec 2021
Cited by 6 | Viewed by 4166
Abstract
Pulmonary arterial hypertension (PAH) patients eventually die of right heart failure (RHF). Currently, there is no suitable pre-clinical model to study PAH. Therefore, we aim to develop a right heart dysfunction (RHD) model using the 3-dimensional engineered heart tissue (EHT) approach and cardiomyocytes [...] Read more.
Pulmonary arterial hypertension (PAH) patients eventually die of right heart failure (RHF). Currently, there is no suitable pre-clinical model to study PAH. Therefore, we aim to develop a right heart dysfunction (RHD) model using the 3-dimensional engineered heart tissue (EHT) approach and cardiomyocytes derived from patient-induced pluripotent stem cells (iPSCs) to unravel the mechanisms that determine the fate of a pressure-overloaded right ventricle. iPSCs from PAH and healthy control subjects were differentiated into cardiomyocytes (iPSC-CMs), incorporated into the EHT, and maintained for 28 days. In comparison with control iPSC-CMs, PAH-derived iPSC-CMs exhibited decreased beating frequency and increased contraction and relaxation times. iPSC-CM alignment within the EHT was observed. PAH-derived EHTs exhibited higher force, and contraction and relaxation times compared with control EHTs. Increased afterload was induced using 2× stiffer posts from day 0. Due to high variability, there were no functional differences between normal and stiffer EHTs, and no differences in the hypertrophic gene expression. In conclusion, under baseline spontaneous conditions, PAH-derived iPSC-CMs and EHTs show prolonged contraction compared with controls, as observed clinically in PAH patients. Further optimization of the hypertrophic model and profound characterization may provide a platform for disease modelling and drug screening. Full article
(This article belongs to the Special Issue Cellular Pathogenesis of Pulmonary Arterial Hypertension)
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11 pages, 4462 KiB  
Article
Incremental Experience in In Vitro Primary Culture of Human Pulmonary Arterial Endothelial Cells Harvested from Swan-Ganz Pulmonary Arterial Catheters
by Birger Tielemans, Leanda Stoian, Allard Wagenaar, Mathias Leys, Catharina Belge, Marion Delcroix and Rozenn Quarck
Cells 2021, 10(11), 3229; https://doi.org/10.3390/cells10113229 - 19 Nov 2021
Cited by 5 | Viewed by 2927
Abstract
Pulmonary arterial hypertension (PAH) is a devastating condition affecting the pulmonary microvascular wall and endothelium, resulting in their partial or total obstruction. Despite a combination of expensive vasodilatory therapies, mortality remains high. Personalized therapeutic approaches, based on access to patient material to unravel [...] Read more.
Pulmonary arterial hypertension (PAH) is a devastating condition affecting the pulmonary microvascular wall and endothelium, resulting in their partial or total obstruction. Despite a combination of expensive vasodilatory therapies, mortality remains high. Personalized therapeutic approaches, based on access to patient material to unravel patient specificities, could move the field forward. An innovative technique involving harvesting pulmonary arterial endothelial cells (PAECs) at the time of diagnosis was recently described. The aim of the present study was to fine-tune the initial technique and to phenotype the evolution of PAECs in vitro subcultures. PAECs were harvested from Swan-Ganz pulmonary arterial catheters during routine diagnostic or follow up right heart catheterization. Collected PAECs were phenotyped by flow cytometry and immunofluorescence focusing on endothelial-specific markers. We highlight the ability to harvest patients’ PAECs and to maintain them for up to 7–12 subcultures. By tracking the endothelial phenotype, we observed that PAECs could maintain an endothelial phenotype for several weeks in culture. The present study highlights the unique opportunity to obtain homogeneous subcultures of primary PAECs from patients at diagnosis and follow-up. In addition, it opens promising perspectives regarding tailored precision medicine for patients suffering from rare pulmonary vascular diseases. Full article
(This article belongs to the Special Issue Cellular Pathogenesis of Pulmonary Arterial Hypertension)
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16 pages, 39626 KiB  
Article
Overexpression of Msx1 in Mouse Lung Leads to Loss of Pulmonary Vessels Following Vascular Hypoxic Injury
by James West, Anandharajan Rathinasabapathy, Xinping Chen, Sheila Shay, Shanti Gladson and Megha Talati
Cells 2021, 10(9), 2306; https://doi.org/10.3390/cells10092306 - 3 Sep 2021
Cited by 2 | Viewed by 2867
Abstract
Pulmonary arterial hypertension (PAH) is a progressive lung disease caused by thickening of the pulmonary arterial wall and luminal obliteration of the small peripheral arteries leading to increase in vascular resistance which elevates pulmonary artery pressure that eventually causes right heart failure and [...] Read more.
Pulmonary arterial hypertension (PAH) is a progressive lung disease caused by thickening of the pulmonary arterial wall and luminal obliteration of the small peripheral arteries leading to increase in vascular resistance which elevates pulmonary artery pressure that eventually causes right heart failure and death. We have previously shown that transcription factor Msx1 (mainly expressed during embryogenesis) is strongly upregulated in transformed lymphocytes obtained from PAH patients, especially IPAH. Under pathological conditions, Msx1 overexpression can cause cell dedifferentiation or cell apoptosis. We hypothesized that Msx1 overexpression contributes to loss of small pulmonary vessels in PAH. In IPAH lung, MSX1 protein localization was strikingly increased in muscularized remodeled pulmonary vessels, whereas it was undetectable in control pulmonary arteries. We developed a transgenic mouse model overexpressing MSX1 (MSX1OE) by about 4-fold and exposed these mice to normoxic, sugen hypoxic (3 weeks) or hyperoxic (100% 02 for 3 weeks) conditions. Under normoxic conditions, compared to controls, MSX1OE mice demonstrated a 30-fold and 2-fold increase in lung Msx1 mRNA and protein expression, respectively. There was a significant retinal capillary dropout (p < 0.01) in MSX1OE mice, which was increased further (p < 0.03) with sugen hypoxia. At baseline, the number of pulmonary vessels in MSX1OE mice was similar to controls. In sugen-hypoxia-treated MSX1OE mice, the number of small (0–25 uM) and medium (25–50 uM) size muscularized vessels increased approximately 2-fold (p < 0.01) compared to baseline controls; however, they were strikingly lower (p < 0.001) in number than in sugen-hypoxia-treated control mice. In MSX1OE mouse lung, 104 genes were upregulated and 67 genes were downregulated compared to controls. Similarly, in PVECs, 156 genes were upregulated and 320 genes were downregulated from siRNA to MSX1OE, and in PVSMCs, 65 genes were upregulated and 321 genes were downregulated from siRNA to MSX1OE (with control in the middle). Many of the statistically significant GO groups associated with MSX1 expression in lung, PVECs, and PVSMCs were similar, and were involved in cell cycle, cytoskeletal and macromolecule organization, and programmed cell death. Overexpression of MSX1 suppresses many cell-cycle-related genes in PVSMCs but induces them in PVECs. In conclusion, overexpression of Msx1 leads to loss of pulmonary vessels, which is exacerbated by sugen hypoxia, and functional consequences of Msx1 overexpression are cell-dependent. Full article
(This article belongs to the Special Issue Cellular Pathogenesis of Pulmonary Arterial Hypertension)
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15 pages, 1259 KiB  
Article
Circulating Cell Biomarkers in Pulmonary Arterial Hypertension: Relationship with Clinical Heterogeneity and Therapeutic Response
by Olga Tura-Ceide, Isabel Blanco, Jéssica Garcia-Lucio, Roberto del Pozo, Agustín Roberto García, Elisabet Ferrer, Isabel Crespo, Diego A. Rodríguez-Chiaradia, Carmen Pilar Simeon-Aznar, Manuel López-Meseguer, Clara Martín-Ontiyuelo, Víctor I. Peinado and Joan Albert Barberà
Cells 2021, 10(7), 1688; https://doi.org/10.3390/cells10071688 - 4 Jul 2021
Cited by 11 | Viewed by 3562
Abstract
Background: Endothelial dysfunction is central to PAH. In this study, we simultaneously analysed circulating levels of endothelial microvesicles (EMVs) and progenitor cells (PCs) in PAH and in controls, as biomarkers of pulmonary endothelial integrity and evaluated differences among PAH subtypes and as a [...] Read more.
Background: Endothelial dysfunction is central to PAH. In this study, we simultaneously analysed circulating levels of endothelial microvesicles (EMVs) and progenitor cells (PCs) in PAH and in controls, as biomarkers of pulmonary endothelial integrity and evaluated differences among PAH subtypes and as a response to treatment. Methods: Forty-seven controls and 144 patients with PAH (52 idiopathic, 9 heritable, 31 associated with systemic sclerosis, 15 associated with other connective tissue diseases, 20 associated with HIV and 17 associated with portal hypertension) were evaluated. Forty-four patients with scleroderma and 22 with HIV infection, but without PAH, were also studied. Circulating levels of EMVs, total (CD31+CD42b−) and activated (CD31+CD42b−CD62E+), as well as circulating PCs (CD34+CD133+CD45low) were measured by flow cytometry and the EMVs/PCs ratio was computed. In treatment-naïve patients, measurements were repeated after 3 months of PAH therapy. Results: Patients with PAH showed higher numbers of EMVs and a lower percentage of PCs, compared with healthy controls. The EMV/PC ratio was increased in PAH patients, and in patients with SSc or HIV without PAH. After starting PAH therapy, individual changes in EMVs and PCs were variable, without significant differences being observed as a group. Conclusion: PAH patients present disturbed vascular homeostasis, reflected in changes in circulating EMV and PC levels, which are not restored with PAH targeted therapy. Combined measurement of circulating EMVs and PCs could be foreseen as a potential biomarker of endothelial dysfunction in PAH. Full article
(This article belongs to the Special Issue Cellular Pathogenesis of Pulmonary Arterial Hypertension)
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21 pages, 3321 KiB  
Article
Right Ventricle Remodeling Metabolic Signature in Experimental Pulmonary Hypertension Models of Chronic Hypoxia and Monocrotaline Exposure
by Thaïs Hautbergue, Fabrice Antigny, Angèle Boët, François Haddad, Bastien Masson, Mélanie Lambert, Amélie Delaporte, Jean-Baptiste Menager, Laurent Savale, Jérôme Le Pavec, Elie Fadel, Marc Humbert, Christophe Junot, François Fenaille, Benoit Colsch and Olaf Mercier
Cells 2021, 10(6), 1559; https://doi.org/10.3390/cells10061559 - 21 Jun 2021
Cited by 17 | Viewed by 4119
Abstract
Introduction: Over time and despite optimal medical management of patients with pulmonary hypertension (PH), the right ventricle (RV) function deteriorates from an adaptive to maladaptive phenotype, leading to RV failure (RVF). Although RV function is well recognized as a prognostic factor of PH, [...] Read more.
Introduction: Over time and despite optimal medical management of patients with pulmonary hypertension (PH), the right ventricle (RV) function deteriorates from an adaptive to maladaptive phenotype, leading to RV failure (RVF). Although RV function is well recognized as a prognostic factor of PH, no predictive factor of RVF episodes has been elucidated so far. We hypothesized that determining RV metabolic alterations could help to understand the mechanism link to the deterioration of RV function as well as help to identify new biomarkers of RV failure. Methods: In the current study, we aimed to characterize the metabolic reprogramming associated with the RV remodeling phenotype during experimental PH induced by chronic-hypoxia-(CH) exposure or monocrotaline-(MCT) exposure in rats. Three weeks after PH initiation, we hemodynamically characterized PH (echocardiography and RV catheterization), and then we used an untargeted metabolomics approach based on liquid chromatography coupled to high-resolution mass spectrometry to analyze RV and LV tissues in addition to plasma samples from MCT-PH and CH-PH rat models. Results: CH exposure induced adaptive RV phenotype as opposed to MCT exposure which induced maladaptive RV phenotype. We found that predominant alterations of arginine, pyrimidine, purine, and tryptophan metabolic pathways were detected on the heart (LV+RV) and plasma samples regardless of the PH model. Acetylspermidine, putrescine, guanidinoacetate RV biopsy levels, and cytosine, deoxycytidine, deoxyuridine, and plasmatic thymidine levels were correlated to RV function in the CH-PH model. It was less likely correlated in the MCT model. These pathways are well described to regulate cell proliferation, cell hypertrophy, and cardioprotection. These findings open novel research perspectives to find biomarkers for early detection of RV failure in PH. Full article
(This article belongs to the Special Issue Cellular Pathogenesis of Pulmonary Arterial Hypertension)
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Review

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18 pages, 454 KiB  
Review
Role of Long Non-Coding RNAs in Pulmonary Arterial Hypertension
by Yun Han, Md Khadem Ali, Kamal Dua, Edda Spiekerkoetter and Yuqiang Mao
Cells 2021, 10(8), 1892; https://doi.org/10.3390/cells10081892 - 26 Jul 2021
Cited by 20 | Viewed by 4574
Abstract
Pulmonary arterial hypertension (PAH) is a debilitating condition of the pulmonary circulatory system that occurs in patients of all ages and if untreated, eventually leads to right heart failure and death. Despite existing medical treatment options that improve survival and quality of life, [...] Read more.
Pulmonary arterial hypertension (PAH) is a debilitating condition of the pulmonary circulatory system that occurs in patients of all ages and if untreated, eventually leads to right heart failure and death. Despite existing medical treatment options that improve survival and quality of life, the disease remains incurable. Thus, there is an urgent need to develop novel therapies to treat this disease. Emerging evidence suggests that long non-coding RNAs (lncRNAs) play critical roles in pulmonary vascular remodeling and PAH. LncRNAs are implicated in pulmonary arterial endothelial dysfunction by modulating endothelial cell proliferation, angiogenesis, endothelial mesenchymal transition, and metabolism. LncRNAs are also involved in inducing different pulmonary arterial vascular smooth muscle cell phenotypes, such as cell proliferation, apoptosis, migration, regulation of the phenotypic switching, and cell cycle. LncRNAs are essential regulators of gene expression that affect various diseases at the chromatin, transcriptional, post-translational, and even post-translational levels. Here, we focus on the role of LncRNAs and their molecular mechanisms in the pathogenesis of PAH. We also discuss the current research challenge and potential biomarker and therapeutic potentials of lncRNAs in PAH. Full article
(This article belongs to the Special Issue Cellular Pathogenesis of Pulmonary Arterial Hypertension)
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24 pages, 1221 KiB  
Review
ROCK Inhibition as Potential Target for Treatment of Pulmonary Hypertension
by Tadeu L. Montagnoli, Jaqueline S. da Silva, Susumu Z. Sudo, Aimeé D. Santos, Gabriel F. Gomide, Mauro P. L. de Sá and Gisele Zapata-Sudo
Cells 2021, 10(7), 1648; https://doi.org/10.3390/cells10071648 - 30 Jun 2021
Cited by 25 | Viewed by 5117
Abstract
Pulmonary hypertension (PH) is a cardiovascular disease caused by extensive vascular remodeling in the lungs, which ultimately leads to death in consequence of right ventricle (RV) failure. While current drugs for PH therapy address the sustained vasoconstriction, no agent effectively targets vascular cell [...] Read more.
Pulmonary hypertension (PH) is a cardiovascular disease caused by extensive vascular remodeling in the lungs, which ultimately leads to death in consequence of right ventricle (RV) failure. While current drugs for PH therapy address the sustained vasoconstriction, no agent effectively targets vascular cell proliferation and tissue inflammation. Rho-associated protein kinases (ROCKs) emerged in the last few decades as promising targets for PH therapy, since ROCK inhibitors demonstrated significant anti-remodeling and anti-inflammatory effects. In this review, current aspects of ROCK inhibition therapy are discussed in relation to the treatment of PH and RV dysfunction, from cell biology to preclinical and clinical studies. Full article
(This article belongs to the Special Issue Cellular Pathogenesis of Pulmonary Arterial Hypertension)
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27 pages, 2391 KiB  
Review
Hydrogen Sulfide Metabolism and Pulmonary Hypertension
by Lukas Roubenne, Roger Marthan, Bruno Le Grand and Christelle Guibert
Cells 2021, 10(6), 1477; https://doi.org/10.3390/cells10061477 - 12 Jun 2021
Cited by 22 | Viewed by 4683
Abstract
Pulmonary hypertension (PH) is a severe and multifactorial disease characterized by a progressive elevation of pulmonary arterial resistance and pressure due to remodeling, inflammation, oxidative stress, and vasoreactive alterations of pulmonary arteries (PAs). Currently, the etiology of these pathological features is not clearly [...] Read more.
Pulmonary hypertension (PH) is a severe and multifactorial disease characterized by a progressive elevation of pulmonary arterial resistance and pressure due to remodeling, inflammation, oxidative stress, and vasoreactive alterations of pulmonary arteries (PAs). Currently, the etiology of these pathological features is not clearly understood and, therefore, no curative treatment is available. Since the 1990s, hydrogen sulfide (H2S) has been described as the third gasotransmitter with plethoric regulatory functions in cardiovascular tissues, especially in pulmonary circulation. Alteration in H2S biogenesis has been associated with the hallmarks of PH. H2S is also involved in pulmonary vascular cell homeostasis via the regulation of hypoxia response and mitochondrial bioenergetics, which are critical phenomena affected during the development of PH. In addition, H2S modulates ATP-sensitive K+ channel (KATP) activity, and is associated with PA relaxation. In vitro or in vivo H2S supplementation exerts antioxidative and anti-inflammatory properties, and reduces PA remodeling. Altogether, current findings suggest that H2S promotes protective effects against PH, and could be a relevant target for a new therapeutic strategy, using attractive H2S-releasing molecules. Thus, the present review discusses the involvement and dysregulation of H2S metabolism in pulmonary circulation pathophysiology. Full article
(This article belongs to the Special Issue Cellular Pathogenesis of Pulmonary Arterial Hypertension)
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24 pages, 2212 KiB  
Review
Progenitor/Stem Cells in Vascular Remodeling during Pulmonary Arterial Hypertension
by France Dierick, Julien Solinc, Juliette Bignard, Florent Soubrier and Sophie Nadaud
Cells 2021, 10(6), 1338; https://doi.org/10.3390/cells10061338 - 28 May 2021
Cited by 27 | Viewed by 6167
Abstract
Pulmonary arterial hypertension (PAH) is characterized by an important occlusive vascular remodeling with the production of new endothelial cells, smooth muscle cells, myofibroblasts, and fibroblasts. Identifying the cellular processes leading to vascular proliferation and dysfunction is a major goal in order to decipher [...] Read more.
Pulmonary arterial hypertension (PAH) is characterized by an important occlusive vascular remodeling with the production of new endothelial cells, smooth muscle cells, myofibroblasts, and fibroblasts. Identifying the cellular processes leading to vascular proliferation and dysfunction is a major goal in order to decipher the mechanisms leading to PAH development. In addition to in situ proliferation of vascular cells, studies from the past 20 years have unveiled the role of circulating and resident vascular in pulmonary vascular remodeling. This review aims at summarizing the current knowledge on the different progenitor and stem cells that have been shown to participate in pulmonary vascular lesions and on the pathways regulating their recruitment during PAH. Finally, this review also addresses the therapeutic potential of circulating endothelial progenitor cells and mesenchymal stem cells. Full article
(This article belongs to the Special Issue Cellular Pathogenesis of Pulmonary Arterial Hypertension)
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22 pages, 3018 KiB  
Review
Iron Deficiency in Pulmonary Arterial Hypertension: A Deep Dive into the Mechanisms
by Marceau Quatredeniers, Pedro Mendes-Ferreira, Diana Santos-Ribeiro, Morad K. Nakhleh, Maria-Rosa Ghigna, Sylvia Cohen-Kaminsky and Frédéric Perros
Cells 2021, 10(2), 477; https://doi.org/10.3390/cells10020477 - 23 Feb 2021
Cited by 34 | Viewed by 6495
Abstract
Pulmonary arterial hypertension (PAH) is a severe cardiovascular disease that is caused by the progressive occlusion of the distal pulmonary arteries, eventually leading to right heart failure and death. Almost 40% of patients with PAH are iron deficient. Although widely studied, the mechanisms [...] Read more.
Pulmonary arterial hypertension (PAH) is a severe cardiovascular disease that is caused by the progressive occlusion of the distal pulmonary arteries, eventually leading to right heart failure and death. Almost 40% of patients with PAH are iron deficient. Although widely studied, the mechanisms linking between PAH and iron deficiency remain unclear. Here we review the mechanisms regulating iron homeostasis and the preclinical and clinical data available on iron deficiency in PAH. Then we discuss the potential implications of iron deficiency on the development and management of PAH. Full article
(This article belongs to the Special Issue Cellular Pathogenesis of Pulmonary Arterial Hypertension)
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