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Pulmonary Hypertension: Far from a Rare Disease—a Journey from Diagnosis...
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Pulmonary Hypertension: Far from a Rare Disease—a Journey from Diagnosis to Treatment

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: 31 August 2026 | Viewed by 3486

Special Issue Editors

Dr. Giovanna Manzi

E-Mail Website
Guest Editor
Department of Clinical, Internal, Anesthesiology and Cardiovascular Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy
Interests: pulmonary arterial hypertension; heart failure
Dr. Silvia Papa

E-Mail Website
Guest Editor
Department of Clinical, Internal, Anesthesiology and Cardiovascular Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy
Interests: pulmonary arterial hypertension; heart failure
Special Issues, Collections and Topics in MDPI journals
Dr. Tommaso Recchioni

E-Mail Website
Guest Editor Assistant
Department of Clinical, Internal, Anesthesiology and Cardiovascular Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy
Interests: pulmonary arterial hypertension; heart failure; arrhythmias

Special Issue Information

Dear Colleagues,

Pulmonary hypertension (PH) is a complex and progressive condition characterized by elevated blood pressure in the pulmonary arteries, leading to significant morbidity and mortality. In recent years, there has been a growing interest in this disease, which requires a precise and accurate diagnostic workup. Avoiding misdiagnosis is essential to avoid prescribing inappropriate and dangerous treatments.

This Special Issue aims at providing a comprehensive overview of the latest advancements in the understanding, diagnosis, and treatment of pulmonary hypertension.

We would like to address different forms of PH, from the most frequent, such as PH associated with left heart disease, to the rarest, such as group 1 PH (pulmonary arterial hypertension). We invite contributions that explore the pathophysiological mechanisms underlying PH, including molecular and cellular pathways, genetic factors, and environmental influences. Manuscripts focusing on innovative diagnostic techniques, such as advanced imaging modalities and biomarker identification, are also welcome. Additionally, we seek papers that address the latest therapeutic strategies, including pharmacological interventions, surgical approaches, and emerging treatments aimed at improving patient outcomes.

This Special Issue will serve as a valuable resource for researchers, clinicians, and healthcare professionals involved in the management of pulmonary hypertension, offering insights into the current state of the field and future directions for research and clinical practice.

Dr. Giovanna Manzi
Dr. Silvia Papa
Guest Editors

Dr. Tommaso Recchioni
Guest Editor Assistant

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 250 words) can be sent to the Editorial Office for assessment.

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 hypertension
  • pathophysiology
  • diagnosis
  • treatment
  • molecular mechanisms
  • cellular pathways
  • genetic factors
  • environmental influences
  • advanced imaging
  • biomarkers

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Published Papers (3 papers)

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Research

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15 pages, 2073 KB  
Article
Prognostic Value of the RVFWLS/PASP Ratio in Pulmonary Arterial Hypertension
by Hongjie Bian, Qinhua Zhao, Fengling Ju, Lan Wang, Yupei Han, Hongling Qiu, Cijun Luo, Pei Gang, Ke Li and Xumeng Ding
J. Cardiovasc. Dev. Dis. 2026, 13(4), 151; https://doi.org/10.3390/jcdd13040151 - 30 Mar 2026
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Abstract
Background: The right ventricular free wall longitudinal strain to pulmonary arterial systolic pressure (RVFWLS/PASP) ratio is an emerging echocardiographic index for evaluating right ventricular–pulmonary artery (RV-PA) coupling. This study aimed to evaluate its prognostic significance and incremental value in risk stratification for patients [...] Read more.
Background: The right ventricular free wall longitudinal strain to pulmonary arterial systolic pressure (RVFWLS/PASP) ratio is an emerging echocardiographic index for evaluating right ventricular–pulmonary artery (RV-PA) coupling. This study aimed to evaluate its prognostic significance and incremental value in risk stratification for patients with pulmonary arterial hypertension (PAH). Methods: We conducted a retrospective–prospective cohort study of 149 adult PAH patients (87 idiopathic PAH and 62 connective tissue disease-associated PAH). RVFWLS was measured via speckle tracking echocardiography, and PASP was estimated using Doppler. The primary endpoint was event-free survival, defined as the first occurrence of all-cause mortality, lung transplantation, or rehospitalization for right heart failure. Kaplan–Meier and multivariate Cox regression analyses were performed to identify independent predictors. Results: During a median follow-up of 32 months, 78 primary events occurred. Patients in the lower RVFWLS/PASP group (<0.246%/mmHg) exhibited significantly worse exercise capacity, higher NT-proBNP levels, and poorer hemodynamics compared with the higher group (≥0.246%/mmHg) (all p < 0.001). The event-free survival rate for the composite endpoint was significantly lower in the group with reduced RVFWLS/PASP compared with that observed in the higher RVFWLS/PASP group (log-rank p < 0.05). Multivariate Cox regression analysis demonstrated RVFWLS/PASP ≥ 0.246%/mmHg was independently predictive of reduced risk for the primary endpoint (HR = 0.46, 95%CI 0.23–0.93, p < 0.05). Moreover, RVFWLS/PASP facilitated additional risk stratification among patients classified as low risk based on established models (FPHN, COMPERA 2.0, and REVEAL Lite 2). Conclusions: RVFWLS/PASP is a robust, independent determinant of long-term prognosis in patients with PAH. As a noninvasive measure of RV-PA coupling, it provides significant incremental value for clinical risk assessment and treatment monitoring. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Far from a Rare Disease—a Journey from Diagnosis to Treatment)
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7 pages, 618 KB  
Communication
Oral Anticoagulants in Chronic Thromboembolic Pulmonary Hypertension: Tradition or Innovation?
by Domenico Laviola, Giovanna Manzi, Tommaso Recchioni, Maria Cristina Luise, Valentina Mercurio, Alexandra Mihai, Roberto Badagliacca, Silvia Papa and Carmine Dario Vizza
J. Cardiovasc. Dev. Dis. 2025, 12(7), 271; https://doi.org/10.3390/jcdd12070271 - 16 Jul 2025
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Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) can complicate the clinical course of patients with acute pulmonary embolism, with a variable prevalence of 0.5–4%. Beyond specific therapeutic strategies, including pulmonary endarterectomy, balloon pulmonary angioplasty and pulmonary vasodilators, lifelong anticoagulation still represents the mainstay of treatment [...] Read more.
Chronic thromboembolic pulmonary hypertension (CTEPH) can complicate the clinical course of patients with acute pulmonary embolism, with a variable prevalence of 0.5–4%. Beyond specific therapeutic strategies, including pulmonary endarterectomy, balloon pulmonary angioplasty and pulmonary vasodilators, lifelong anticoagulation still represents the mainstay of treatment for this condition. The main historical experience supports the use of vitamin K antagonists (VKAs) in CTEPH patients; conversely, the efficacy and safety of direct oral anticoagulants (DOACs) in this setting are unclear. Growing experience, mainly from small studies and registries, is improving our knowledge, showing that DOACs may represent a valid and promising alternative to warfarin in CTEPH patients. Therefore, in the management of cases with a newly diagnosed CTEPH, clinicians are very often in the difficult position of (a) having to choose which anticoagulant to initiate in anticoagulant-naïve patients or (b) having to evaluate whether it is necessary to switch to a VKA in patients already on DOACs. This article aims to critically summarize the current evidence comparing DOACs and VKAs in CTEPH, discussing their efficacy and safety profiles and exploring their clinical applicability. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Far from a Rare Disease—a Journey from Diagnosis to Treatment)
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Review

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43 pages, 3854 KB  
Review
The New Era of Pulmonary Hypertension: The Dawn of Disease Modification & Therapeutic Modalities
by Noyan Ramazani, Lacey Barnes, Alex Wong, Divyansh Sharma, Aditi Singh and KaChon Lei
J. Cardiovasc. Dev. Dis. 2026, 13(5), 174; https://doi.org/10.3390/jcdd13050174 - 22 Apr 2026
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Abstract
Pulmonary hypertension (PH) can be defined as a mean pulmonary artery pressure (mPAP) greater than 20 mm Hg at rest during right heart catheterization (RHC). The reported prevalence of PH throughout the globe has been estimated to impact approximately 1% of the total [...] Read more.
Pulmonary hypertension (PH) can be defined as a mean pulmonary artery pressure (mPAP) greater than 20 mm Hg at rest during right heart catheterization (RHC). The reported prevalence of PH throughout the globe has been estimated to impact approximately 1% of the total population, with a majority of those afflicted being women more than men. Numerous etiologies give rise to the pathophysiology of PH, including heart disease (i.e., left-sided heart failure), lung diseases, and other unclear causes related to chronic stages and complications surrounding long-standing pulmonary thromboembolisms, side effects of certain medications, and genetic and environmental factors. Untreated PH can lead to severe morbidities such as cardio-renal syndrome and congestive hepatopathy (cardiac cirrhosis). Management of PH focuses on decreasing pulmonary pressures by using vasodilators such as prostanoids, and phosphodiesterase type 5 (PDE-5) inhibitors, as well as newer treatments such as sotatercept, which inhibits activin signaling, thereby inhibiting excessive cell growth in the pulmonary artery vasculature and down-regulating the pro-proliferative pathways. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Far from a Rare Disease—a Journey from Diagnosis to Treatment)
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