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Clinical Advances in Pulmonary Heart Disease
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Clinical Advances in Pulmonary Heart Disease

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Cardiovascular Medicine".

Deadline for manuscript submissions: 20 January 2025 | Viewed by 4815

Special Issue Editors

Dr. Alexandra D. Arvanitaki

E-Mail Website
Guest Editor
1. First Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, 546 36 Thessaloniki, Greece
2. National Pulmonary Hypertension Service, Royal Brompton Hospital, London SW3 6NP, UK
Interests: pulmonary hypertension; congenital heart disease; artificial intelligence
Special Issues, Collections and Topics in MDPI journals
Dr. Stephen John Wort

E-Mail Website
Guest Editor
National Pulmonary Hypertension Service, Royal Brompton Hospital, London SW3 6NP, UK
Interests: lung disease; interstitial lung disease; pulmonary hypertension

Special Issue Information

Dear Colleagues, 

Pulmonary hypertension (PH) represents a rare and heterogeneous group of pulmonary vasculopathies defined by elevated mean pulmonary arterial pressure and pulmonary vascular resistance that leads progressively to right heart failure and premature death, if not early diagnosed and promptly treated. PH is haemodynamically defined as a mean pulmonary artery pressure > 20 mmHg and is classified into five distinct groups, taking into account the underlying condition, clinical and haemodynamic features PH can be attributed to a wide spectrum of underlying conditions, such as left heart disease, lung disease, pulmonary artery obstruction, but it can also be associated with drugs and toxins, connective tissue disease (CTD-PAH), HIV infection, portal hypertension (portal-PAH), congenital heart disease (CHD-PAH) and schistosomiasis or it can be idiopathic (IPAH) or heritable (HPAH). In the past 30 years, there was an increased research interest in the field of pathophysiology, diagnosis and targeted pulmonary arterial hypertension (PAH) therapies. New diagnostic modalities and algorithms were employed to detect PH early, while new drugs have been developed that target different pathophysiologic pathways aiming to increase life expectancy. In this Special Issue, we would like to invite original clinical and basic research, meta-analyses, and state-of-the-art reviews related to clinical advances in the whole spectrum of PH and pulmonary heart disease in general. We will be grateful to receive your submissions to move the field of PH forward.

Dr. Alexandra D. Arvanitaki
Dr. Stephen John Wort
Guest Editors

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 Clinical Medicine is an international peer-reviewed open access semimonthly 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 2600 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 heart disease
  • pulmonary hypertension
  • diagnosis
  • treatment
  • clinical advances

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Published Papers (3 papers)

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Research

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10 pages, 670 KiB  
Article
Predictors of Mortality in Pulmonary Hypertension-Associated Chronic Lung Disease
by Jay Pescatore, Matthew Bittner, Gilbert D’Alonzo, Sheila Weaver and Shameek Gayen
J. Clin. Med. 2024, 13(12), 3472; https://doi.org/10.3390/jcm13123472 - 14 Jun 2024
Viewed by 1019
Abstract
Background/Objectives: Pulmonary hypertension (PH) often accompanies chronic lung diseases. Several chronic lung diseases with PH portends unfavorable outcomes. We investigated which variables in this cohort of patients with chronic lung disease and PH predicts mortality. Methods: This is a retrospective analysis [...] Read more.
Background/Objectives: Pulmonary hypertension (PH) often accompanies chronic lung diseases. Several chronic lung diseases with PH portends unfavorable outcomes. We investigated which variables in this cohort of patients with chronic lung disease and PH predicts mortality. Methods: This is a retrospective analysis of patients with chronic lung disease and PH at a single tertiary, academic center. The underlying lung disease included were COPD, IPF, other fibrotic ILD, non-fibrotic ILD, fibrotic sarcoidosis, and CPFE. All patients had right heart catheterization diagnostic of PH as well as pulmonary function testing data including 6 min walk testing. Univariable and multivariate Cox regression was performed to identify variables associated with mortality. Results: We identified 793 patients with chronic lung disease and PH. In total, 144 patients died prior to potential lung transplant. In multivariable Cox regression IPF, other fibrotic ILD, non-fibrotic ILD, and CPFE were significantly associated with an increased risk of mortality. Severe PH (PVR > 5 WU), FEV1 < 30% predicted, FVC < 40% predicted, 6 min walk distance < 150 m were also significantly associated with an increased risk of mortality. Conclusions: Carrying a diagnosis of IPF, CPFE, fibrotic ILD, or non-fibrotic ILD with PH has an increased risk of mortality as compared to COPD with PH. Hemodynamic, PVR > 5 WU, 6 min walk test less than 150 m, as well as spirometric data including FEV1 < 30% and FVC < 40% predicted were independently associated with an increased risk of death. Full article
(This article belongs to the Special Issue Clinical Advances in Pulmonary Heart Disease)
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Review

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20 pages, 552 KiB  
Review
Towards a Better Prognosis in Patients with Systemic Sclerosis-Related Pulmonary Arterial Hypertension: Recent Developments and Perspectives
by Maria Boutel, Athanasia Dara, Alexandra Arvanitaki, Cleopatra Deuteraiou, Maria Mytilinaiou and Theodoros Dimitroulas
J. Clin. Med. 2024, 13(19), 5834; https://doi.org/10.3390/jcm13195834 - 30 Sep 2024
Viewed by 1340
Abstract
Precapillary pulmonary hypertension (PH) is a significant complication of systemic sclerosis (SSc). It represents one of the leading causes of morbidity and mortality, correlating with a significantly dismal prognosis and quality of life. Despite advancements in the management of patients with pulmonary arterial [...] Read more.
Precapillary pulmonary hypertension (PH) is a significant complication of systemic sclerosis (SSc). It represents one of the leading causes of morbidity and mortality, correlating with a significantly dismal prognosis and quality of life. Despite advancements in the management of patients with pulmonary arterial hypertension associated with SSc (SSc-PAH), no significant improvement has been reported in survival of patients with precapillary SSc-PH associated with extensive lung parenchyma disease. International expert consensus and guidelines for the management of PH recommend annual screening of SSc patients for early detection of pre-capillary PH. The implementation of screening algorithms capable of identifying patients with a high likelihood of developing PH could help limit unnecessary right-heart catheterization procedures and prevent significant delay in diagnosis. Furthermore, early initiation of up-front combination targeted therapy in patients with PAH has shown increase in survival rates, indicating that timely and aggressive medical therapy is key for stabilizing and even improving functional class, hemodynamic parameters and 6 min walking distance (6MWD) in this population. Further research is warranted into the benefit of PAH-targeted therapies in patients with PH associated with lung disease. Lastly, we discuss the potential role of immunosuppression using biologic agents in the therapeutic management of precapillary PH in SSc patients. Full article
(This article belongs to the Special Issue Clinical Advances in Pulmonary Heart Disease)
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22 pages, 778 KiB  
Review
Endothelial Function in Pulmonary Arterial Hypertension: From Bench to Bedside
by Michele Correale, Francesco Chirivì, Ester Maria Lucia Bevere, Lucia Tricarico, Michele D’Alto, Roberto Badagliacca, Natale D. Brunetti, Carmine Dario Vizza and Stefano Ghio
J. Clin. Med. 2024, 13(8), 2444; https://doi.org/10.3390/jcm13082444 - 22 Apr 2024
Cited by 1 | Viewed by 1750
Abstract
Pulmonary arterial hypertension is a complex pathology whose etiology is still not completely well clarified. The pathogenesis of pulmonary arterial hypertension involves different molecular mechanisms, with endothelial dysfunction playing a central role in disease progression. Both individual genetic predispositions and environmental factors seem [...] Read more.
Pulmonary arterial hypertension is a complex pathology whose etiology is still not completely well clarified. The pathogenesis of pulmonary arterial hypertension involves different molecular mechanisms, with endothelial dysfunction playing a central role in disease progression. Both individual genetic predispositions and environmental factors seem to contribute to its onset. To further understand the complex relationship between endothelial and pulmonary hypertension and try to contribute to the development of future therapies, we report a comprehensive and updated review on endothelial function in pulmonary arterial hypertension. Full article
(This article belongs to the Special Issue Clinical Advances in Pulmonary Heart Disease)
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