New Perspectives on Pulmonary Hypertension (PH)

A special issue of Journal of Cardiovascular Development and Disease (ISSN 2308-3425). This special issue belongs to the section "Acquired Cardiovascular Disease".

Deadline for manuscript submissions: closed (31 January 2024) | Viewed by 8092

Special Issue Editor

Dr. Nika Skoro-sajer
E-Mail Website
Guest Editor
Department of Medicine II, Division of Cardiology, Medical University of Vienna, 1090 Vienna, Austria
Interests: pulmonary arterial hypertension; chronic thromboembolic pulmonary hypertension

Special Issue Information

Dear Colleagues,

Guidelines summarize and evaluate evidence and should facilitate decision making for health professionals. Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions and be associated with a variety of cardiovascular and respiratory diseases. The management of PH patients is complex, requiring a multifaceted, holistic, and multidisciplinary approach. Still, there are some unanswered topics which should be discussed.

  • New hemodynamic definition of PH and its clinical implications
  • How to detect PH earlier
  • What is new in the treatment algorithm of PH
  • How to treat PH associated with left heart disease (PH-LHD)
  • CTEPH—ways to cure pulmonary hypertension
  • PH centers
  • New standards

The aim of this Special Issue is to provide the readers of JCDD with original publications, allowing better knowledge of PH and sharing the latest findings in the field.

Dr. Nika Skoro-sajer
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

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. Journal of Cardiovascular Development and Disease is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • pulmonary arterial hypertension
  • hemodynamic definition

Published Papers (7 papers)

Order results
Result details
Select all
Export citation of selected articles as:

Research

Jump to: Review, Other

13 pages, 2042 KiB  
Article
Novel lncRNA LNC_000113 Drives the Activation of Pulmonary Adventitial Fibroblasts through Modulating PTEN/Akt/FoxO1 Pathway
J. Cardiovasc. Dev. Dis. 2023, 10(6), 262; https://doi.org/10.3390/jcdd10060262 - 15 Jun 2023
Viewed by 1006
Abstract
The activation of pulmonary adventitial fibroblasts (PAFs) is one of the key components of pulmonary arterial remodelling in pulmonary arterial hypertension (PAH). Emerging evidence indicates that lncRNAs may play fibrotic roles in a range of diseases. In this present study, we identified a [...] Read more.
The activation of pulmonary adventitial fibroblasts (PAFs) is one of the key components of pulmonary arterial remodelling in pulmonary arterial hypertension (PAH). Emerging evidence indicates that lncRNAs may play fibrotic roles in a range of diseases. In this present study, we identified a novel lncRNA, LNC_000113, in pulmonary adventitial fibroblasts (PAFs) and characterised its role in the Galectin-3-induced activation of PAFs in rats. Galectin-3 led to elevated expression of lncRNA LNC_000113 in PAFs. The expression of this lncRNA was primarily PAF enriched. A progressive increase in lncRNA LNC_000113 expression was observed in rats with monocrotaline (MCT)-induced PAH rats. Knockdown of lncRNA LNC_000113 cancelled the Galectin-3′s fibroproliferative effect on PAFs and prevented the transition of fibroblasts to myofibroblasts. The loss-of-function study demonstrated that lncRNA LNC_000113 activated PAFs through the PTEN/Akt/FoxO1 pathway. These results propose lncRNA LNC_000113 drives the activation of PAFs and promotes fibroblast phenotypic alterations. Full article
(This article belongs to the Special Issue New Perspectives on Pulmonary Hypertension (PH))
Show Figures

Figure 1

14 pages, 4019 KiB  
Article
Sympathetic Denervation and Pharmacological Stimulation of Parasympathetic Nervous System Prevent Pulmonary Vascular Bed Remodeling in Rat Model of Chronic Thromboembolic Pulmonary Hypertension
J. Cardiovasc. Dev. Dis. 2023, 10(2), 40; https://doi.org/10.3390/jcdd10020040 - 23 Jan 2023
Viewed by 1402
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) develops in 1.5–2.0% of patients experiencing pulmonary embolism (PE) and is characterized by stable pulmonary artery obstruction, heart failure, and poor prognosis. Little is known about involvement of autonomic nervous system (ANS) in the mechanisms of CTEPH. This [...] Read more.
Chronic thromboembolic pulmonary hypertension (CTEPH) develops in 1.5–2.0% of patients experiencing pulmonary embolism (PE) and is characterized by stable pulmonary artery obstruction, heart failure, and poor prognosis. Little is known about involvement of autonomic nervous system (ANS) in the mechanisms of CTEPH. This study was aimed at evaluation of the effect of vagal and sympathetic denervation, as well as stimulation of the parasympathetic nervous system, on the outcomes of CTEPH in rats. CTEPH was induced by multiple intravenous injections of alginate microspheres. Sympathetic and vagal denervation was performed using unilateral surgical ablation of the stellate ganglion and vagotomy, respectively. Stimulation of the parasympathetic nervous system was carried out by administering pyridostigmine. The effect of neuromodulatory effects was assessed in terms of hemodynamics, histology, and gene expression. The results demonstrated the key role of ANS in the development of CTEPH. Sympathetic denervation as well as parasympathetic stimulation resulted in attenuated pulmonary vascular remodeling. These salutary changes were associated with altered MMP2 and TIMP1 expression in the lung and decreased FGFb level in the blood. Unilateral vagotomy had no effect on physiological and morphological outcomes of the study. The data obtained contribute to the identification of new therapeutic targets for CTEPH treatment. Full article
(This article belongs to the Special Issue New Perspectives on Pulmonary Hypertension (PH))
Show Figures

Figure 1

20 pages, 3376 KiB  
Article
A Story of PA/BSA and Biomarkers to Diagnose Pulmonary Hypertension in Patients with Severe Aortic Valve Stenosis—The Rise of IGF-BP2 and GDF-15
J. Cardiovasc. Dev. Dis. 2023, 10(1), 22; https://doi.org/10.3390/jcdd10010022 - 05 Jan 2023
Cited by 1 | Viewed by 1668
Abstract
(1) Background: Currently, echocardiography is the primary non-invasive diagnostic method used to screen patients with severe aortic valve stenosis (AS) for pulmonary hypertension (PH) by estimating systolic pulmonary artery pressure (sPAP). Other radiological methods have been a focus of research in the past [...] Read more.
(1) Background: Currently, echocardiography is the primary non-invasive diagnostic method used to screen patients with severe aortic valve stenosis (AS) for pulmonary hypertension (PH) by estimating systolic pulmonary artery pressure (sPAP). Other radiological methods have been a focus of research in the past couple of years, as it was shown that by determining the pulmonary artery (PA) diameter, prognostic statements concerning overall mortality could be made in these patients. This study compared established and novel cardiovascular biomarkers with the PA/BSA value to detect PH in patients with severe AS. (2) Methods: The study cohort comprised 188 patients with severe AS undergoing transcatheter aortic valve replacement (TAVR), who were then divided into two groups based on PA/BSA values obtained through CT-angiography. The presence of PH was defined as a PA/BSA ≥ 16.6 mm/m2 (n = 81), and absence as a PA/BSA < 16.6 mm/m2 (n = 107). Blood samples were taken before TAVR to assess cardiovascular biomarkers used in this study, namely brain natriuretic peptide (BNP), cardiac troponin I (cTnI), high-sensitive troponin (hsTN), soluble suppression of tumorigenesis-2 (sST2), growth/differentiation factor 15 (GDF-15), heart-type fatty acid-binding protein (H-FABP), insulin-like growth factor binding protein 2 (IGF-BP2), and soluble urokinase-type plasminogen activator receptor (suPAR). (3) Results: Patients with a PA/BSA ≥ 16.6 mm/m2 showed significantly higher levels of BNP (p = <0.001), GDF-15 (p = 0.040), and H-FABP (p = 0.007). The other investigated cardiovascular biomarkers did not significantly differ between the two groups. To predict a PA/BSA ≥ 16.6 mm/m2, cut-off values for the biomarkers were calculated. Here, GDF-15 (p = 0.029; cut-off 1172.0 pg/mL) and BNP (p < 0.001; cut-off 2194.0 pg/mL) showed significant results. Consequently, analyses of combined biomarkers were performed, which yielded IGF-BP2 + BNP (AUC = 0.721; 95%CI = 0.585–0.857; p = 0.004) as the best result of the two-way analyses and GDF-15 + IGF-BP2 + BNP (AUC = 0.727; 95%CI = 0.590–0.864; p = 0.004) as the best result of the three-way analyses. No significant difference regarding the 1-year survival between patients with PA/BSA < 16.6 mm/m2 and patients with PA/BSA ≥ 16.6 mm/m2 was found (log-rank test: p = 0.452). (4) Conclusions: Although PA/BSA aims to reduce the bias of the PA value caused by different body compositions and sizes, it is still a controversial parameter for diagnosing PH. Combining the parameter with different cardiovascular biomarkers did not lead to a significant increase in the diagnostic precision for detecting PH in patients with severe AS. Full article
(This article belongs to the Special Issue New Perspectives on Pulmonary Hypertension (PH))
Show Figures

Graphical abstract

Review

Jump to: Research, Other

12 pages, 618 KiB  
Review
Updated Clinical Classification and Hemodynamic Definitions of Pulmonary Hypertension and Its Clinical Implications
J. Cardiovasc. Dev. Dis. 2024, 11(3), 78; https://doi.org/10.3390/jcdd11030078 - 27 Feb 2024
Viewed by 135
Abstract
Pulmonary hypertension (PH) refers to a pathologic elevation of the mean pulmonary artery pressure (mPAP) and is associated with increased morbidity and mortality in a wide range of medical conditions. These conditions are classified according to similarities in pathophysiology and management in addition [...] Read more.
Pulmonary hypertension (PH) refers to a pathologic elevation of the mean pulmonary artery pressure (mPAP) and is associated with increased morbidity and mortality in a wide range of medical conditions. These conditions are classified according to similarities in pathophysiology and management in addition to their invasive hemodynamic profiles. The 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension present the newest clinical classification system and includes significant updates to the hemodynamic definitions. Pulmonary hypertension is now hemodynamically defined as an mPAP > 20 mmHg, reduced from the previous threshold of ≥25 mmHg, due to important insights from both normative and prognostic data. Pulmonary vascular resistance has been extended into the definition of pre-capillary pulmonary hypertension, with an updated threshold of >2 Wood Units (WU), to help differentiate pulmonary vascular disease from other causes of increased mPAP. Exercise pulmonary hypertension has been reintroduced into the hemodynamic definitions and is defined by an mPAP/cardiac output slope of >3 mmHg/L/min between rest and exercise. While these new hemodynamic thresholds will have a significant impact on the diagnosis of pulmonary hypertension, no evidence-based treatments are available for patients with mPAP between 21–24 mmHg and/or PVR between 2–3 WU or with exercise PH. This review highlights the evidence underlying these major changes and their implications on the diagnosis and management of patients with pulmonary hypertension. Full article
(This article belongs to the Special Issue New Perspectives on Pulmonary Hypertension (PH))
Show Figures

Figure 1

11 pages, 758 KiB  
Review
Unrecognized Pulmonary Hypertension in Non-Cardiac Surgical Patients: At-Risk Populations, Preoperative Evaluation, Intraoperative Management and Postoperative Complications
J. Cardiovasc. Dev. Dis. 2023, 10(9), 403; https://doi.org/10.3390/jcdd10090403 - 19 Sep 2023
Viewed by 1077
Abstract
Pulmonary hypertension is a well-established independent risk factor for perioperative complications after elective non-cardiac surgery. Patients undergoing cardiac surgery are routinely evaluated for the presence of pulmonary hypertension in the preoperative period. Better monitoring in the postoperative critical care setting leads to more [...] Read more.
Pulmonary hypertension is a well-established independent risk factor for perioperative complications after elective non-cardiac surgery. Patients undergoing cardiac surgery are routinely evaluated for the presence of pulmonary hypertension in the preoperative period. Better monitoring in the postoperative critical care setting leads to more efficient management of potential complications. Data among patients with pulmonary hypertension undergoing elective non-cardiac surgery are scant. Moreover, the condition may be unidentified at the time of surgery. Also, monitoring after non-cardiac surgery can be very limited in the PACU setting, as opposed to the critical care setting. All these factors can result in a higher postoperative complication rate and poor outcomes. Full article
(This article belongs to the Special Issue New Perspectives on Pulmonary Hypertension (PH))
Show Figures

Figure 1

16 pages, 2285 KiB  
Review
Lung Transplantation for Pulmonary Arterial Hypertension: Optimized Referral and Listing Based on an Evolving Disease Concept
J. Cardiovasc. Dev. Dis. 2023, 10(8), 350; https://doi.org/10.3390/jcdd10080350 - 15 Aug 2023
Viewed by 1284
Abstract
Pulmonary hypertension (PH) was once a devastating and fatal disease entity, the outlook of which has been significantly improved by the continued progress of medical treatment algorithms. However, some patients still ultimately fail to achieve an adequate clinical response despite receiving maximal medical [...] Read more.
Pulmonary hypertension (PH) was once a devastating and fatal disease entity, the outlook of which has been significantly improved by the continued progress of medical treatment algorithms. However, some patients still ultimately fail to achieve an adequate clinical response despite receiving maximal medical treatment. Historically, lung transplantation (LTx) has been the only effective therapeutic option that could lead to satisfactory outcomes and save these advanced patients’ lives. However, patients with PH tend to have the highest mortality rates on the transplant waiting list; especially after comprehensive medical treatment, they continue to deteriorate very rapidly, eventually missing optimal transplantation windows. Balancing optimized medical treatment with the appropriate timing of referral and listing has been highly controversial in LTx for patients with PH. The 2021 consensus document for the selection of lung transplant candidates from the International Society for Heart and Lung Transplantation (ISHLT) updated the specific recommendations for the LTx referral and listing time for patients with PH based on objective risk stratification. Herein, we review the evolving PH-related concepts and highlight the optimization of LTx referral and listing for patients with PH, as well as their management on the waiting list. Full article
(This article belongs to the Special Issue New Perspectives on Pulmonary Hypertension (PH))
Show Figures

Figure 1

Other

Jump to: Research, Review

9 pages, 518 KiB  
Brief Report
Intravenous Treprostinil in Severe Inoperable Chronic Thromboembolic Pulmonary Hypertension Using Implantable Pumps—Single-Center Experience over More Than a Decade
J. Cardiovasc. Dev. Dis. 2023, 10(8), 318; https://doi.org/10.3390/jcdd10080318 - 27 Jul 2023
Viewed by 909
Abstract
The management of chronic thromboembolic pulmonary hypertension has significantly changed over the last decade with the availability of both specific therapies and interventional treatments. In parallel, implantable pumps for intravenous administration of treprostinil have broadened the spectrum of continuous prostanoid infusion. We evaluated [...] Read more.
The management of chronic thromboembolic pulmonary hypertension has significantly changed over the last decade with the availability of both specific therapies and interventional treatments. In parallel, implantable pumps for intravenous administration of treprostinil have broadened the spectrum of continuous prostanoid infusion. We evaluated the course of 17 consecutive patients with inoperable chronic thromboembolic pulmonary hypertension treated with treprostinil by means of an implantable infusion pump between 2011 and 2023 at our center. Complications associated with the infusion system were rare, leading to 0.4 unplanned surgical interventions during 17,160 patient days. No additional safety signals were detected, and clinical benefits achieved with subcutaneous treprostinil before pump implantation could be maintained in all patients. No catheter-related infections or thromboembolic events were observed. Implantable infusion pumps offer an attractive alternative to subcutaneous treprostinil for patients intolerant to the subcutaneous route, including those with chronic thromboembolic pulmonary hypertension. Full article
(This article belongs to the Special Issue New Perspectives on Pulmonary Hypertension (PH))
Show Figures

Figure 1

Back to TopTop