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The Past, Present, and Future of Pulmonary Hypertension: Pathophysiology and...
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The Past, Present, and Future of Pulmonary Hypertension: Pathophysiology and Treatment Prospects

A Special Issue of Journal of Cardiovascular Development and Disease (ISSN 2308-3425) belonging to the section "Cardiovascular Clinical Research".

Deadline for manuscript submissions: 30 November 2026 | Viewed by 5195

Editors

Dr. Roxana Sulica

E-Mail Website
Guest Editor
Department of Medicine, Pulmonary Critical Care and Sleep Division, New York University Grossman School of Medicine, New York University Langone Health, New York, NY 10016, USA
Interests: pulmonary hypertension
Special Issues, Collections and Topics in MDPI journals
Dr. Himanshu Deshwal

E-Mail Website
Guest Editor
Pulmonary Hypertension Clinic, Section of Pulmonary, Sleep and Critical Care Medicine, Department of Medicine, West Virginia University School of Medicine, Morgantown, WV 26505, USA
Interests: pulmonary hypertension; pulmonary vascular disorders; right ventricular failure; hemodynamics; point-of-care ultrasonography; pulmonary embolism
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Pulmonary hypertension (PH) remains a complex and evolving disorder involving the pulmonary vasculature. This Special Issue explores the historical milestones that shaped our foundational understanding of PH, recent advances in diagnostic and therapeutic strategies, and future directions in treatment approaches.

This collection aims to provide a comprehensive overview of the changing landscape of PH. Topics may include translational genetics and metabolomics in PH physiology, current challenges in classification and risk stratification, and emerging therapies—from novel drug targets to precision medicine approaches. We invite contributions that reflect the depth of PH research, with the goal of inspiring continued innovation in improving outcomes for patients with this life-threatening condition.

Dr. Roxana Sulica
Dr. Himanshu Deshwal
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 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-anonymized 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
  • exercise-induced pulmonary hypertension
  • sotatercept
  • precision-medicine
  • BMPR2
  • prostacyclin

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Published Papers (5 papers)

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Research

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13 pages, 2578 KB  
Article
Combination of TAPSE/sPAP Ratio and Myocardial Work to Assess Prognosis in Patients with Pulmonary Arterial Hypertension
by Jian Wang, Yingying Xu, Zhenwei Li and Hanbin Cui
J. Cardiovasc. Dev. Dis. 2026, 13(7), 324; https://doi.org/10.3390/jcdd13070324 - 10 Jul 2026
Viewed by 781
Abstract
Objective: Pulmonary arterial hypertension (PAH) is a progressive disease leading to right ventricular (RV) hypertrophy and failure. This study aims to evaluate the prognostic value of combining the tricuspid annular plane systolic excursion/systolic pulmonary artery pressure (TAPSE/sPAP) ratio with right ventricular myocardial work [...] Read more.
Objective: Pulmonary arterial hypertension (PAH) is a progressive disease leading to right ventricular (RV) hypertrophy and failure. This study aims to evaluate the prognostic value of combining the tricuspid annular plane systolic excursion/systolic pulmonary artery pressure (TAPSE/sPAP) ratio with right ventricular myocardial work (RVMW) parameters in patients with PAH, to improve early risk stratification. Methods: A total of 43 PAH patients diagnosed via right heart catheterization were enrolled. Echocardiography-derived TAPSE/sPAP ratio and RVMW parameters, including right ventricular global work efficiency (RVGWE), global work index (RVGWI), global constructive work (RVGCW), and global wasted work (RVGWW), were measured. Clinical worsening events were recorded during a median 515-day follow-up. Statistical analyses included correlation tests, Firth penalized logistic regression, receiver operating characteristic (ROC) curves, and Kaplan–Meier survival analysis. Results: The TAPSE/sPAP ratio correlated negatively with RVGCW (r = −0.346, p = 0.023) and RVGWW (r = −0.417, p = 0.005), but not with RVGWE or RVGWI. Clinical worsening events occurred in 25.6% of patients, with significantly lower TAPSE/sPAP ratio (0.16 vs. 0.24 mm/mmHg), RVGWE (72.0% vs. 88.5%), and RVGWI (431.0 vs. 641.0 mmHg%) in the Event group (all p < 0.05). Multivariate Firth penalized logistic regression was used for combining TAPSE/sPAP ratio with RVGWE. ROC analysis demonstrated that the combination of TAPSE/sPAP and RVGWE yielded superior predictive power (AUC = 0.949, p < 0.001) compared to individual parameters. Conclusion: Non-invasive assessment of TAPSE/sPAP ratio and RVGWE provides significant prognostic value in PAH. Their combination enhances early risk prediction, offering a practical tool for clinical management. Full article
(This article belongs to the Special Issue The Past, Present, and Future of Pulmonary Hypertension: Pathophysiology and Treatment Prospects)
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13 pages, 2631 KB  
Article
ANO1 (TMEM16A) Genetic Variants, Promoter Methylation, and Chloride Dysregulation in Pulmonary Hypertension
by İrfan Yaman, Hasan Korkmaz, Arzu Etem Akağaç, Tuğçe Kaymaz, Rauf Önder and Ebru Etem Önalan
J. Cardiovasc. Dev. Dis. 2026, 13(6), 283; https://doi.org/10.3390/jcdd13060283 - 22 Jun 2026
Viewed by 654
Abstract
Background: Pulmonary arterial hypertension (PAH) is a rare and progressive disorder characterized by increased pulmonary vascular resistance and vascular remodeling. Genetic polymorphisms, epigenetic modifications, and ion channel dysregulation are increasingly recognized as key contributors to disease pathogenesis. Anoctamin-1 (ANO1/TMEM16A), a calcium-activated chloride channel, [...] Read more.
Background: Pulmonary arterial hypertension (PAH) is a rare and progressive disorder characterized by increased pulmonary vascular resistance and vascular remodeling. Genetic polymorphisms, epigenetic modifications, and ion channel dysregulation are increasingly recognized as key contributors to disease pathogenesis. Anoctamin-1 (ANO1/TMEM16A), a calcium-activated chloride channel, plays a critical role in vascular tone regulation. Objective: This study aimed to investigate the association between ANO1 gene polymorphisms (rs7127129 and rs2509153), promoter methylation status, and serum chloride levels in patients with idiopathic pulmonary arterial hypertension (IPAH), congenital heart disease (CHD), and chronic thromboembolic pulmonary hypertension (CTEPH). Methods: A total of 106 IPAH patients, 40 CHD patients, and 30 CTEPH patients, together with 125 healthy controls, were included. The control group had a comparable age distribution, with a balanced sex ratio, whereas females predominated in all three PH groups. Genotyping was performed using TaqMan-based real-time PCR. Promoter methylation was analyzed using bisulfite conversion followed by quantitative real-time PCR. Serum chloride levels were measured using an ion-selective electrode method. Results: No significant association was observed between rs7127129 and rs2509153 polymorphisms and IPAH or CTEPH (p > 0.05). However, rs7127129 showed a significant association with CHD (p < 0.05). After excluding hypertensive patients, both polymorphisms remained significantly associated with CHD. Serum chloride levels differed significantly among groups (p < 0.001), with higher levels observed particularly in the CTEPH and CHD groups compared to controls, while IPAH patients exhibited intermediate but still elevated levels relative to controls. In contrast, promoter methylation levels were significantly lower in all patient groups compared to controls. An inverse relationship between chloride levels and methylation status was observed. Conclusions: ANO1 polymorphisms are not major determinants of IPAH or CTEPH but may contribute to CHD susceptibility. Increased serum chloride levels, together with decreased promoter methylation, suggest a potential mechanistic link between ion channel dysregulation and epigenetic alterations in pulmonary hypertension. Further large-scale and functional studies are warranted. Full article
(This article belongs to the Special Issue The Past, Present, and Future of Pulmonary Hypertension: Pathophysiology and Treatment Prospects)
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14 pages, 548 KB  
Article
Real-World Outcomes of Inhaled Treprostinil in Pulmonary Hypertension Related to Interstitial Lung Disease: A Multicenter, Retrospective Analysis
by Andrew R. Kyle, Arun Jose, Kristen Catherman, Jean Elwing, Roxana Sulica, Gerald S. Zavorsky and Namita Sood
J. Cardiovasc. Dev. Dis. 2026, 13(3), 129; https://doi.org/10.3390/jcdd13030129 - 10 Mar 2026
Viewed by 2718
Abstract
Inhaled Treprostinil is the primary treatment of pulmonary hypertension related to interstitial lung disease (PH-ILD). Despite treatment effectiveness in clinical trials, the real-world safety and tolerability of this therapy remains unclear. We conducted a multicenter, retrospective review of adults with PH-ILD who were [...] Read more.
Inhaled Treprostinil is the primary treatment of pulmonary hypertension related to interstitial lung disease (PH-ILD). Despite treatment effectiveness in clinical trials, the real-world safety and tolerability of this therapy remains unclear. We conducted a multicenter, retrospective review of adults with PH-ILD who were prescribed inhaled treprostinil. We assessed clinical outcomes, 6 min walk distance (6MWD) and changes in natriuretic peptides (BNP, NT-proBNP), as well as medication tolerance. Eighty-three patients met the inclusion criteria. The 6MWD data was collected but a limited number of patients had results within close proximity to initiation of inhalational treprostinil with only seven patients having assessments within the 3 months prior to initiation as well as 3 months post therapy. Limited 6MWD data is likely due, in part, to coinciding with the COVID pandemic, limiting face-to-face interactions and exercise testing. The majority of our subjects, 63%, had an absolute improvement in their BNP level, over a mean duration of 170 days. However, no significant difference was detected between baseline and follow-up natriuretic peptide levels. Adherence was assessed and the majority (77%) of patients remained on therapy at the time of censoring, with three-quarters (75%) meeting the target dose. Of the 15 patients intolerant to nebulized treprostinil who were transitioned to a dry powder inhaler, the majority (87%) were able to tolerate the other formulation. The medication was well-tolerated with a large percentage of patients remaining on therapy indefinitely and reaching the targeted therapeutic dose. Full article
(This article belongs to the Special Issue The Past, Present, and Future of Pulmonary Hypertension: Pathophysiology and Treatment Prospects)
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Review

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22 pages, 5339 KB  
Review
Multimodal Management of Chronic Thromboembolic Pulmonary Hypertension: A Narrative Review of Treatment Selection and Sequencing
by Houda Gharsalli, Mohammed Alshahrani, Olfa Harbi, Nawal Alwadai, Abdalla M. Al-Asiri, Fatmah Alahmari, Amal Alqahtani, Najla Al-Jahash, Lobna Abbag, Saud Alqahtani and Khaled Abdulwahab Amer
J. Cardiovasc. Dev. Dis. 2026, 13(9), 433; https://doi.org/10.3390/jcdd13090433 - 3 Sep 2026
Viewed by 275
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a treatable yet frequently underdiagnosed late complication of acute pulmonary embolism, in which incompletely resolved thromboemboli organise into fibrotic obstructions of the pulmonary arteries and, together with a secondary small-vessel arteriopathy affecting both obstructed and non-obstructed territories, [...] Read more.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a treatable yet frequently underdiagnosed late complication of acute pulmonary embolism, in which incompletely resolved thromboemboli organise into fibrotic obstructions of the pulmonary arteries and, together with a secondary small-vessel arteriopathy affecting both obstructed and non-obstructed territories, raise pulmonary vascular resistance, impose right ventricular pressure overload, and—if left untreated—progress to right heart failure and death. Three established therapies address the disease at distinct anatomical levels: pulmonary endarterectomy, the potentially curative treatment of choice for surgically accessible disease; balloon pulmonary angioplasty, for distal inoperable lesions or residual disease; and pulmonary hypertension-targeted medical therapy, for the accompanying microvasculopathy. Their evaluation in randomised controlled trials and large registries has produced a multimodal paradigm in which these treatments are combined and sequenced according to operability and lesion distribution. This narrative review synthesises evidence published through January 2026 to accomplish the following: (i) summarise and critically appraise the contemporary evidence for each modality, distinguishing guideline-supported recommendations from observational, proof-of-concept, and expert-opinion-based practice; (ii) describe how treatments are selected, sequenced, and bridged in relation to operability and to residual or recurrent pulmonary hypertension after endarterectomy; and (iii) situate treatment within the broader chronic thromboembolic pulmonary disease spectrum and the case-finding pathway after pulmonary embolism. Rather than proposing a validated algorithm, it emphasises that, because each modality carries distinct indications and risks, decisions should be individualised through multidisciplinary assessment at expert centres, with early referral central to optimising outcomes. Full article
(This article belongs to the Special Issue The Past, Present, and Future of Pulmonary Hypertension: Pathophysiology and Treatment Prospects)
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Other

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12 pages, 1806 KB  
Brief Report
Parenteral Treprostinil as an Effective Bridge to Lung Transplant in Patients with Severe Pulmonary Hypertension Associated with Interstitial Lung Disease
by Jennifer Hopkins and Shameek Gayen
J. Cardiovasc. Dev. Dis. 2026, 13(9), 460; https://doi.org/10.3390/jcdd13090460 - 12 Sep 2026
Viewed by 129
Abstract
Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is associated with substantial morbidity and mortality. Lung transplantation remains the definitive therapy for selected patients; however, severe pulmonary vascular dysfunction and hemodynamic instability may complicate transplant candidacy and pre-transplant management. The role of parenteral [...] Read more.
Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is associated with substantial morbidity and mortality. Lung transplantation remains the definitive therapy for selected patients; however, severe pulmonary vascular dysfunction and hemodynamic instability may complicate transplant candidacy and pre-transplant management. The role of parenteral prostacyclin therapy as a bridge to transplantation in this population remains poorly defined. The primary objective of this retrospective case series was to evaluate the utility of parenteral treprostinil as a bridge therapy for lung transplantation in 14 patients with severe PH-ILD from 2018 to 2025. Secondary objectives were to describe subsequent changes in clinical outcomes, including hemodynamic parameters measured by right heart catheterization, echocardiographic findings, the six-minute walk distance, oxygen requirements, the need for veno-arterial extracorporeal membrane oxygenation (VA-ECMO), and successful lung transplantation. Parenteral treprostinil was associated with significant improvements in pulmonary artery systolic pressure, mean pulmonary artery pressure, pulmonary vascular resistance, and BNP levels, while pulmonary capillary wedge pressure, cardiac output, cardiac index, and oxygen requirements remained stable following therapy initiation. Echocardiography demonstrated improvement in right ventricular dilation and right ventricular dysfunction. Most patients were successfully bridged to lung transplantation, while only two patients died prior to transplantation. In selected patients with severe PH-ILD, parenteral treprostinil may improve pulmonary hemodynamics and facilitate a successful bridge to lung transplantation without worsening oxygenation. Full article
(This article belongs to the Special Issue The Past, Present, and Future of Pulmonary Hypertension: Pathophysiology and Treatment Prospects)
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