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Novel Therapeutic Approaches for Pulmonary Hypertension

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

Deadline for manuscript submissions: closed (30 November 2023) | Viewed by 21494

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Special Issue Editor

Prof. Dr. Marcin Kurzyna

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Guest Editor

Department of Pulmonary Circulation, Thromboembolic Diseases and Cardiology, Centre of Postgraduate Medical Education, European Health Center Otwock, Otwock, Poland
Interests: pulmonary hypertension; pulmonary embolism; pulmonary arterial hypertension; chronic thromboembolic pulmonary hypertension; balloon pulmonary angioplasty; echocardiography

Special Issue Information

Dear Colleagues,

Over the past 25 years, tremendous progress has been made in the therapeutic possibilities for pulmonary hypertension. This statement applies to both pharmacological and surgical or percutaneous interventions. The goals and methods of therapy differ significantly depending on the etiology of pulmonary hypertension. In group I, according to current ESC/ERS Guidelines (pulmonary arterial hypertension, PAH), the introduction of epoprostenol and other drugs from the targeted therapy at the turn of the 20th and 21st centuries changed the "primary pulmonary hypertension" from a clearly fatal condition to more chronic disease. The following years brought numerous studies of new drugs and their combinations to obtain the optimal therapeutic effect. Interventional methods such as atrial septostomy, Pott's anastomosis, and pulmonary artery denervation are also utilized to reduce right ventricular failure. Lung transplantation remains the ultimate treatment for PAH, but the timing of listing for this procedure is still not clearly defined. Drugs that proved successful in treating PAH were tested in patients with post-capillary pulmonary hypertension (group II) and pulmonary hypertension associated with chronic pulmonary diseases (group III). The results of these studies are inconclusive and do not clearly indicate which patients benefit from PAH-like therapy. In contrast, many interventions that control the underlying heart failure or chronic pulmonary disease have beneficial effects on pulmonary artery pressure and the degree of right ventricular failure. Balloon pulmonary angioplasty and modern pharmacological treatment have been added to pulmonary endarterectomy, the recognized treatment option for chronic thromboembolic pulmonary hypertension. It gave an unprecedented possibility of treatment which is tailored to the individual subject. Research on novel therapeutic approaches is still underway and goes beyond the well-known canons of management.

In the present Special Issue, we want to gather a collection of original, interesting and innovative papers on new therapeutic options for pulmonary hypertension. We invite authors of papers in the field of clinical medicine in the adult and pediatric populations and basic science researchers who work on experimental studies facilitating new therapeutic options. Let this Special Issue be a forum for the exchange of fresh ideas and innovative concepts!

Prof. Dr. Marcin Kurzyna
Guest Editor

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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 hypertension
Pulmonary arterial hypertension
Chronic thromboembolic pulmonary hypertension
Congenital heart disease
Congestive heart failure
Balloon pulmonary angioplasty
Atrial septostomy
Pulmonary artery denervation
Lung transplantation

Published Papers (5 papers)

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Research

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13 pages, 616 KiB

Open AccessArticle

Association of Electrocardiographic Signs of Right Ventricular Hypertrophy and Clot Localization in Chronic Thromboembolic Pulmonary Hypertension

by Sylwia Sławek-Szmyt, Aleksander Araszkiewicz, Stanisław Jankiewicz, Anna Smukowska-Gorynia, Marek Grygier, Magdalena Janus, Maciej Lesiak and Tatiana Mularek-Kubzdela

J. Clin. Med. 2022, 11(3), 625; https://doi.org/10.3390/jcm11030625 - 26 Jan 2022

Cited by 7 | Viewed by 2290

Abstract

The role of electrocardiography (ECG) in chronic thromboembolic pulmonary hypertension (CTEPH) diagnosis and prognosticating has not been yet established. We aimed to assess the relationships of the recommended ECG criteria of right ventricular hypertrophy (RVH) with clot localization in CTEPH patients. ECG patterns of RVH according to the American College of Cardiology Foundation were assessed in patients with newly diagnosed CTEPH. We enrolled 58 (45.3%) patients with proximal and 70 (54.7%) with distal CTEPH. Receiver-operating characteristics curves analysis indicated that the following ECG abnormalities predicted proximal CTEPH localization: R_V1 > 6 mm—AUC 0.75 (CI: 0.66–0.84, p < 0.00001); S_V6 > 3 mm—AUC 0.70 (CI: 0.60–0.79, p < 0.00001); S_I > R_I wave—AUC 0.67 (CI: 0.58–0.77, p = 0.0004); R_V1:S_V1 > 1.0—AUC 0.66 (CI: 0.56–0.76, p = 0.0009); R_V1 peak > 0.035 s (QRS < 120 ms)—AUC 0.66 (CI: 0.56–0.75, p = 0.0016); R_V1:S_V1 > R_V3(V4):S_V3(V4)—AUC-0.65 (CI: 0.54–0.75, p = 0.0081); R_aVR > 4 mm—AUC 0.62 (CI: 0.52–0.71, p = 0.002) and P_II > 2.5 mm—AUC 0.62 (CI: 0.52–0.72, p = 0.00162). Pulmonary vascular resistance significantly correlated with amplitudes of R_V1 (r = 0.34, p = 0.008), S_V6 (r = 0.53, p = 0.000027) and P_II (r = 0.44, p = 0.00007). In patients with CTEPH, only 8 out of 23 ECG RVH criteria were useful for differentiating between proximal and distal CTEPH localization and we found that R_V1 and S_V6 may contribute as potential discriminators. Full article

(This article belongs to the Special Issue Novel Therapeutic Approaches for Pulmonary Hypertension)

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13 pages, 1693 KiB

Open AccessArticle

Mean Velocity of the Pulmonary Artery as a Clinically Relevant Prognostic Indicator in Patients with Heart Failure with Preserved Ejection Fraction

by Blanca Trejo-Velasco, Ignacio Cruz-González, Manuel Barreiro-Pérez, Elena Díaz-Peláez, Pilar García-González, Ana Martín-García, Rocío Eiros, Soraya Merchán-Gómez, Candelas Pérez del Villar, Oscar Fabregat-Andrés, Francisco Ridocci-Soriano and Pedro L. Sánchez

J. Clin. Med. 2022, 11(3), 491; https://doi.org/10.3390/jcm11030491 - 19 Jan 2022

Cited by 2 | Viewed by 1872

Abstract

Background: Right ventricular (RV) to pulmonary circulation (PC) coupling can stratify prognosis in heart failure (HF). In this study, we assessed the prognostic role of the mean velocity of the pulmonary artery (mvPA) determined by cardiac magnetic resonance (CMR) in HF with preserved ejection fraction (HFpEF). Methods: Inclusion of 58 HFpEF outpatients that underwent CMR with measurement of RV–PC coupling parameters including mvPA between 2016 and 2019. The primary combined endpoint was a composite of HF readmissions and all-cause mortality. Results: Optimal cut-off value of mvPA calculated by receiver operating curve for the prediction of the primary endpoint was 9 cm/s. Over a median follow-up of 23 months (interquartile range: 24), 21 patients met the primary endpoint. The primary endpoint was more frequent in patients with mvPA ≤ 9 cm/s, as indicated by Kaplan–Meier survival curves; Log-Rank: 9.193, p = 0.02, regardless of RV dysfunction. On Cox multivariate analysis, mvPA ≤ 9 cm/s emerged as an independent prognostic predictor of the primary endpoint (HR: 4.11, 95% CI: 1.28–13.19, p = 0.017), together with left atrial area by CMR (HR: 1.08, 95% CI: 1.01–1.24, p = 0.034). Conclusions: In our HFpEF cohort, mvPA was associated with a higher rate of the primary endpoint, regardless of RV function, thus enabling identification of patients at higher risk of cardiovascular events before structural damage onset. Full article

(This article belongs to the Special Issue Novel Therapeutic Approaches for Pulmonary Hypertension)

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22 pages, 29509 KiB

Open AccessArticle

Selective Segmental Pulmonary Angiography: Anatomical, Technical and Safety Aspects of a Must-Learn Technique in Times of Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension

by Maite Velázquez, Nicolás Maneiro, Ana Lareo, Agustín Albarrán, Sergio Huertas, Allende P. Olazábal, Juan F. Delgado, Sergio Alonso, Fernando Sarnago, Julio García Tejada, Fernando Arribas and Pilar Escribano

J. Clin. Med. 2021, 10(15), 3358; https://doi.org/10.3390/jcm10153358 - 29 Jul 2021

Cited by 5 | Viewed by 10174

Abstract

With the advent of balloon pulmonary angioplasty (BPA) for non-surgical chronic thromboembolic pulmonary hypertension (CTEPH) patients, there is renewed interest in the pulmonary angiography technique. This technique is still the standard imaging modality to confirm CTEPH, which, in addition, helps to determine the most appropriate treatment. Furthermore, learning this technique fulfills two main purposes: to identify BPA candidates and to provide the operator with the catheter handling needed to perform BPA. Operators interested in performing BPA must learn not only the pulmonary arteries’ anatomy, but also which are the best angiographic projections and the most suitable catheters to canalize and display each segmental branch. Unfortunately, this information is scarce in the literature. With this goal, learning the diagnostic pulmonary angiography technique can be a first step on the way to perform BPA. Although there are descriptions on how to perform a pulmonary angiography with balloon-tipped catheters and the digital subtraction technique, this technique does not provide operators with the catheter knowledge and manual skill needed to cannulate each segmental branch. In contrast, learning the conventional selective segmental pulmonary angiography (SSPA) technique provides the operator with this knowledge and skills. In this review, based on the experience of the authors, we describe the pulmonary arteries’ anatomy and detail the practical aspects of the SSPA procedure, with the aim of providing operators with the anatomical and technical knowledge needed to perform BPA. We also summarize the contemporary complications of SSPA in CTEPH patients at a reference center. Full article

(This article belongs to the Special Issue Novel Therapeutic Approaches for Pulmonary Hypertension)

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13 pages, 1741 KiB

Open AccessArticle

Balloon Pulmonary Angioplasty in Technically Operable and Technically Inoperable Chronic Thromboembolic Pulmonary Hypertension

by Szymon Darocha, Aleksander Araszkiewicz, Marcin Kurzyna, Marta Banaszkiewicz, Stanisław Jankiewicz, Anna Dobosiewicz, Sylwia Sławek-Szmyt, Magdalena Janus, Maciej Grymuza, Arkadiusz Pietrasik, Tatiana Mularek-Kubzdela, Piotr Kędzierski, Radosław Pietura, Dariusz Zieliński, Andrzej Biederman, Maciej Lesiak and Adam Torbicki

J. Clin. Med. 2021, 10(5), 1038; https://doi.org/10.3390/jcm10051038 - 3 Mar 2021

Cited by 20 | Viewed by 2835

Abstract

Background: In this study, we aimed to assess the efficacy and safety of balloon pulmonary angioplasty (BPA) in patients with technically inoperable distal-type chronic thromboembolic pulmonary hypertension (d-CTEPH) and technically operable proximal-type disease (p-CTEPH) by analyzing the results of BPA treatment in two collaborating CTEPH referral centers. Methods and results: We assessed hemodynamic results, functional efficacy, complication and survival rate after BPA treatment in 70 CTEPH patients (median age 64 years; (interquartile range (IQR): 52–73 years)), of whom 16 (median age 73 years; (QR 62–82 years)) were in the p-CTEPH subgroup. Altogether, 377 BPA procedures were performed, resulting in significant (p < 0.001) improvement in mean pulmonary artery pressure (mPAP 48.6 ± 10 vs. 31.3 ± 8.6 mmHg), pulmonary vascular resistance (694 ± 296 vs. 333 ± 162 dynes*s*cm⁻⁵), six-minute walk test (365 ± 142 vs. 433 ± 120 metres) and N-terminal pro B-type natriuretic peptide (1307 (510–3294) vs. 206 (83–531) pg/mL). The rate of improvement did not differ between the sub-groups. Lung injury episodes and severe hemoptysis were similarly infrequent in d-CTEPH and p-CTEPH (6.4% vs. 5%; p = 0.55 and 1.0% vs. 2.5; p = 0.24, respectively). There was no significant difference between the sub-groups regarding survival (p = 0.53 by log-rank test). Conclusion: BPA may be beneficial in patients with p-CTEPH who cannot undergo pulmonary endarterectomy (PEA). Larger long-term studies are needed to better define the efficacy, safety, and optimal BPA procedural standards in this population. Full article

(This article belongs to the Special Issue Novel Therapeutic Approaches for Pulmonary Hypertension)

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Review

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12 pages, 289 KiB

Open AccessReview

Exercise Training as a Non-Pharmacological Therapy for Patients with Pulmonary Arterial Hypertension: Home-Based Rehabilitation Program and Training Recommendations

by Mariusz Wojciuk, Zofia Dzięcioł-Anikiej, Katarzyna Kaniewska, Mariusz Ciołkiewicz, Diana Moskal-Jasińska and Anna Kuryliszyn-Moskal

J. Clin. Med. 2022, 11(23), 6932; https://doi.org/10.3390/jcm11236932 - 24 Nov 2022

Cited by 4 | Viewed by 2162

Abstract

Pulmonary arterial hypertension (PAH) is a chronic and progressive disorder with a poor prognosis associated with non-specific symptoms, including general weakness, shortness of breath on exertion, and decreased muscle strength and endurance. Despite recent significant progress in the field of PAH therapy, many patients are still characterized by a dynamic course of the disease, a significant reduction in physical performance, a constantly deteriorating quality of life, and limited activity in everyday life. Thus, the main goal of PAH therapy is to ensure an acceptable level of quality of life as early as possible in the course of the disease, reduce the progression of symptoms and, if possible, improve the prognosis, which is still poor. The perception of the importance of activity and exercise has changed significantly in recent years, and rehabilitation dedicated to PAH patients is now considered to be one of the new adjuvant treatment options. Currently, there is insufficient data on what form, frequency, and intensity of exercise are required for the best results. Nevertheless, exercise training (ET) is necessary in order to reverse the accompanying PAH impairment of exercise capacity and, without additional clinical risk, to maximize the benefits of pharmacotherapy. This review summarizes the current state of knowledge on the rehabilitation of PAH patients and presents the available rehabilitation models. In addition, it includes a ready-to-use, illustrated, safe home rehabilitation program with recommendations for its use. Utilizing ET as an adjuvant treatment option to improve the functional capacity and quality of life of patients may enhance the clinical effectiveness of therapeutic management and contribute to the improvement of the quality of care for patients suffering from PAH. The beneficial effect of exercise training on the development of symptoms improves the clinical course of the disease, and a lower incidence of adverse events can lead to a reduction in health care expenditure. Full article

(This article belongs to the Special Issue Novel Therapeutic Approaches for Pulmonary Hypertension)

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