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Pulmonary Hypertension: Current Status of Diagnosis and Treatment
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Pulmonary Hypertension: Current Status of Diagnosis and Treatment

A special issue of Journal of Cardiovascular Development and Disease (ISSN 2308-3425).

Deadline for manuscript submissions: 25 May 2025 | Viewed by 9595

Special Issue 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 is a condition in which blood pressure in the arteries of the lungs (pulmonary arteries) is abnormally high. In pulmonary hypertension, the right side of the heart has to push blood hard through the pulmonary arteries. Over time, right ventricular hypertrophy enlarges and develops cor pulmonale, leading to right-sided heart failure. In the past 20 years, more and more people have focused on the diagnosis and treatment of pulmonary hypertension.

The diagnosis of pulmonary hypertension is obtained mainly through blood tests and imaging methods, such as echocardiography, right heart catheterization, chest X-ray, and so on. Treatment of pulmonary hypertension may include drug therapy, complex percutaneous interventions or surgery, and various other approaches. According to the clinical, neurohormonal, imaging and hemodynamic parameters, it is important to carry out risk stratification for patients to manage the decision-making process. In recent years, the survival rate of pulmonary hypertension patients has improved significantly. However, many clinical questions still remain and even more and more are emerging.

This Special Issue aims to provide an open forum for those trying to solve these questions, whether by reporting their research or by providing comments for review articles. We look forward to receiving your submissions.

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 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 hypertension
  • pulmonary arterial hypertension 
  • diagnosis 
  • treatment 
  • hemodynamics

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Published Papers (4 papers)

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12 pages, 799 KiB  
Article
Nitroprusside Combined with Leg Raise at the Time of Right Heart Catheterization to Differentiate Precapillary from Other Hemodynamic Forms of Pulmonary Hypertension: A Single-Center Pilot Study
by Mostafa Naguib, Ahmed Aljwaid, Dean Marella, Raul J. Flores and Abhishek Singh
J. Cardiovasc. Dev. Dis. 2024, 11(4), 124; https://doi.org/10.3390/jcdd11040124 - 19 Apr 2024
Viewed by 1285
Abstract
Pulmonary hypertension (PH) can arise from several distinct disease processes, with a percentage presenting with combined pre- and postcapillary pulmonary hypertension (cpcPH). Patients with cpcPH are unsuitable candidates for PH-directed therapies due to elevated pulmonary capillary wedge pressures (PCWPs); however, the PCWP is [...] Read more.
Pulmonary hypertension (PH) can arise from several distinct disease processes, with a percentage presenting with combined pre- and postcapillary pulmonary hypertension (cpcPH). Patients with cpcPH are unsuitable candidates for PH-directed therapies due to elevated pulmonary capillary wedge pressures (PCWPs); however, the PCWP is dynamic and is affected by both preload and afterload. Many patients that are diagnosed with cpcPH are hypertensive at the time of right heart catheterization which has the potential to increase the PCWP and, therefore, mimic a more postcapillary-predominant phenotype. In this small pilot study, we examine the effect of nitroprusside combined with dynamic preload augmentation with a passive leg raise maneuver in hypertensive cpcPH patients at the time of right heart catheterization to identify a more precapillary-dominant PH phenotype. Patients that met the criteria of PCWP ≤ 15 mmHg with nitroprusside infusion and PCWP ≤ 18 mmHg with nitroprusside infusion and simultaneous leg raise were started on pulmonary vascular-targeted therapy. Long-term PH therapy was well tolerated, with increased six-minute walk distance, improved WHO functional class, decreased NT-proBNP, and improved REVEAL 2.0 Lite Risk Score in this precapillary-dominant PH phenotype. This small study highlights the importance of characterizing patient physiology beyond resting conditions at the time of right heart catheterization. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Current Status of Diagnosis and Treatment)
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12 pages, 912 KiB  
Article
Pulmonary Vasodilator Therapy Is Associated with Decreased Mortality in Patients with Chronic Lung Disease and Severe Pulmonary Hypertension
by Olivia Schanz, Gerard J. Criner, Parth Rali and Shameek Gayen
J. Cardiovasc. Dev. Dis. 2024, 11(3), 89; https://doi.org/10.3390/jcdd11030089 - 8 Mar 2024
Viewed by 1829
Abstract
The mortality benefit of PAH-specific therapy for patients with pulmonary hypertension (PH) associated with lung disease is not clear. Our aim was to determine whether pulmonary arterial hypertension (PAH)-specific therapy is associated with reduced mortality among all patients with PH associated with lung [...] Read more.
The mortality benefit of PAH-specific therapy for patients with pulmonary hypertension (PH) associated with lung disease is not clear. Our aim was to determine whether pulmonary arterial hypertension (PAH)-specific therapy is associated with reduced mortality among all patients with PH associated with lung disease and in patients with chronic lung disease and severe PH. This was a retrospective cohort study of patients at our institution with chronic lung disease and PH. Survival analysis was performed by comparing patients who received PAH-specific therapy with patients who did not receive pulmonary vasodilators in the entire cohort and in a subgroup of patients with severe PH defined as PVR > 5 WU. We identified 783 patients with chronic lung disease and PH; 246 patients met the new criteria for severe PH. In the entire cohort, a similar survival probability was seen between the treated and untreated PH groups (logrank p = 0.67). In the severe PH subgroup, patients treated with PAH-specific therapy had increased survival probability (logrank p = 0.03). PAH-specific therapy was independently and significantly associated with decreased mortality in severe PH (HR 0.31, 95% CI 0.11–0.88, p = 0.03). PAH-specific therapy may confer a mortality benefit in patients with chronic lung disease and severe PH, which is now defined as PVR > 5 WU, similarly to those with pulmonary arterial hypertension. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Current Status of Diagnosis and Treatment)
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15 pages, 4204 KiB  
Systematic Review
Pulmonary Vasodilator Therapy in Severe Pulmonary Hypertension Due to Chronic Obstructive Pulmonary Disease (Severe PH-COPD): A Systematic Review and Meta-Analysis
by Ahmed Elkhapery, M. Bakri Hammami, Roxana Sulica, Hemanth Boppana, Zeinab Abdalla, Charoo Iyer, Hazem Taifour, Chengu Niu and Himanshu Deshwal
J. Cardiovasc. Dev. Dis. 2023, 10(12), 498; https://doi.org/10.3390/jcdd10120498 - 16 Dec 2023
Cited by 3 | Viewed by 2934
Abstract
Background: Chronic obstructive pulmonary disease-associated pulmonary hypertension (PH-COPD) results in a significant impact on symptoms, quality of life, and survival. There is scant and conflicting evidence about the use of pulmonary hypertension (PH) specific therapy in patients with PH-COPD. Study Design and Methods: [...] Read more.
Background: Chronic obstructive pulmonary disease-associated pulmonary hypertension (PH-COPD) results in a significant impact on symptoms, quality of life, and survival. There is scant and conflicting evidence about the use of pulmonary hypertension (PH) specific therapy in patients with PH-COPD. Study Design and Methods: PubMed, OVID, CINAHL, Cochrane, Embase, and Web of Science were searched using various MESH terms to identify randomized controlled trials (RCTs) or observational studies investigating PH-specific therapies in patients with severe PH-COPD, defined by mean pulmonary artery pressure (mPAP) of more than 35 mm Hg or pulmonary vascular resistance (PVR) of more than 5 woods units on right heart catheterization. The primary outcome was a change in mPAP and PVR. Secondary outcomes were changes in six-minute walk distance (6MWD), changes in the brain-natriuretic peptide (BNP), New York Heart Association (NYHA) functional class, oxygenation, and survival. Results: Thirteen studies satisfied the inclusion criteria, including a total of 328 patients with severe PH-COPD. Out of these, 308 patients received some type of specific therapy for PH. There was a significant reduction in mPAP (mean difference (MD) −3.68, 95% CI [−2.03, −5.32], p < 0.0001) and PVR (MD −1.40 Wood units, 95% CI [−1.97, −0.82], p < 0.00001). There was a significant increase in the cardiac index as well (MD 0.26 L/min/m2, 95% CI [0.14, 0.39], p < 0.0001). There were fewer patients who had NYHA class III/lV symptoms, with an odds ratio of 0.55 (95% CI [0.30, 1.01], p = 0.05). There was no significant difference in the 6MWD (12.62 m, 95% CI [−8.55, 33.79], p = 0.24), PaO2 (MD −2.20 mm Hg, 95% CI [−4.62, 0.22], p = 0.08), or BNP or NT-proBNP therapy (MD −0.15, 95% CI [−0.46, 0.17], p = 0.36). Conclusion: The use of PH-specific therapies in severe PH-COPD resulted in a significant reduction in mPAP and PVR and increased CI, with fewer patients remaining in NYHA functional class III/IV. However, no significant difference in the 6MWD, biomarkers of right ventricular dysfunction, or oxygenation was identified, demonstrating a lack of hypoxemia worsening with treatment. Further studies are needed to investigate the use of PH medications in patients with severe PH-COPD. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Current Status of Diagnosis and Treatment)
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10 pages, 1382 KiB  
Systematic Review
Effect of Obesity on Mortality in Pulmonary Hypertension—A Systematic Review and Meta-Analysis
by Raju Reddy, Saminder Singh Kalra, Bashar Alzghoul, Akram Khan and Yazan Zayed
J. Cardiovasc. Dev. Dis. 2023, 10(10), 419; https://doi.org/10.3390/jcdd10100419 - 6 Oct 2023
Viewed by 2593
Abstract
Obesity is reported to have a protective effect on mortality in pulmonary hypertension (PH), a phenomenon known as obesity paradox. However, the data are conflicting, with some studies showing decreased mortality while other studies found no effect of obesity on mortality. Therefore, we [...] Read more.
Obesity is reported to have a protective effect on mortality in pulmonary hypertension (PH), a phenomenon known as obesity paradox. However, the data are conflicting, with some studies showing decreased mortality while other studies found no effect of obesity on mortality. Therefore, we performed a systematic review and meta-analysis to examine whether there is an association between obesity and mortality in PH. Only patients with PH diagnosed by right heart catheterization were included. We also performed a sub-group analysis of subjects with pre-capillary PH only. A total of six studies met the inclusion criteria, with a sample size of 13,987 patients. Obese subjects had lower mortality compared to non-obese subjects in the combined pre- and post-capillary PH group (hazard ratio 0.79, 95% CI 0.66–0.95, p = 0.01). While obesity was associated with reduction in mortality in the pre-capillary PH group (hazard ratio 0.77, 95% CI 0.60 to 0.98, p = 0.03), this was not uniform across all studies. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Current Status of Diagnosis and Treatment)
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