Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
3.6
3.6
Journals
Diagnostics
Special Issues
Pulmonary Hypertension: Diagnosis and Management
share announcement

Pulmonary Hypertension: Diagnosis and Management

A special issue of Diagnostics (ISSN 2075-4418).

Deadline for manuscript submissions: closed (31 January 2021) | Viewed by 62372

Special Issue Editors

Prof. Dr. Adam Torbicki

E-Mail
Guest Editor
Department of Pulmonary Circulation, Thromboembolic Disease and Cadiology Center of Postgraduate Medical Education, ECZ-Otwock, Otwock, Poland
Prof. Dr. Stephan Rosenkranz

E-Mail Website
Guest Editor
UOC Division of Cardiology, Pneumology, Angiology and Intensive Care| University of Cologne, Cologne, Germany

Special Issue Information

Once neglected, pulmonary hypertension (PH) now attracts increasing numbers of clinicians and researchers. This is due to its high prevalence, nonspecific symptoms and signs, as well as limitations of noninvasive diagnostic methods. Moreover, we all face problems related to the correct assignment of our patients to one of the five PH clinical subgroups, which is crucial for the identification of those in need of specific therapy. Management of PH  may include targeted pharmacotherapy, sophisticated percutaneous interventions or surgery, as well as various combination and hybrid approaches. However, despite the progress that we are witnessing, many clinical problems remain and even more are emerging. The Editors of this Special Issue are grateful for the open forum that was made available by Diagnostics to all who want to help identify and try to resolve these problems, either by reporting their research results, presenting instructive clinical cases, or sharing their experience and knowledge by providing opinion and review articles. We are ready to post the articles on-line as soon as they are accepted after peer-review, the contributions dealing with PH in the time of COVID-19 pandemics will benefit from a special fast-track. An important contribution to this Special Issue has been declared by several teams collaborating within European Reference Network providing cross-border expertise in rare diseases - in this case ERN-LUNG-Pulmonary Hypertension. This is a form of their contribution to European collaboration in this important area. The Editors also promise two special articles—on historical milestones in understanding pulmonary circulation and on deep phenotyping as the future of diagnosis and monitoring PH patients. You are welcome to join our task force preparing this Special Issue on pulmonary hypertension.

Prof. Dr. Adam Torbicki

Prof. Dr. Stephan Rosenkranz
Guest Editor

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. Diagnostics 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 circulation
  • Pulmonary embolism
  • Right ventricular failure
  • Pulmonary arterial hypertension
  • Chronic thromboembolic pulmonary hypertension.

Published Papers (18 papers)

Download All Papers
Order results
Result details
Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:

Editorial

Jump to: Research, Review, Other

2 pages, 178 KiB  
Editorial
Pulmonary Hypertension: Diagnosis and Management
by Adam Torbicki
Diagnostics 2021, 11(6), 1066; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics11061066 - 09 Jun 2021
Cited by 1 | Viewed by 1612
Abstract
With great satisfaction, we now share with you the contents of this Special Issue of Diagnostics dedicated to Diagnosis and Treatment of Pulmonary Hypertension (PH) [...] Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)

Research

Jump to: Editorial, Review, Other

13 pages, 3459 KiB  
Article
Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties
by Marta Pérez Núñez, Sergio Alonso Charterina, Carmen Pérez-Olivares, Yolanda Revilla Ostolaza, Rafael Morales Ruiz, Ana Belén Enguita Valls, Jair Antonio Tenorio, Natalia Gallego Zazo, Alicia De Pablo Gafas, Pablo Lapunzina, Adriana Rodríguez Chaverri and Pilar Escribano Subías
Diagnostics 2021, 11(1), 141; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics11010141 - 19 Jan 2021
Cited by 7 | Viewed by 2334
Abstract
Pulmonary veno-occlusive disease (PVOD) is a very infrequent form of pulmonary arterial hypertension with an aggressive clinical course, poor response to specific vasodilator treatment, and low survival. Confirming a definitive diagnosis is essential to guide treatment and assess lung transplantation. However, in the [...] Read more.
Pulmonary veno-occlusive disease (PVOD) is a very infrequent form of pulmonary arterial hypertension with an aggressive clinical course, poor response to specific vasodilator treatment, and low survival. Confirming a definitive diagnosis is essential to guide treatment and assess lung transplantation. However, in the absence of histological or genetic confirmation, the diagnosis is complex, requiring a clinical suspicion. Multidetector computed tomography (MDCT) is an essential part of the non-invasive diagnostic tools of PVOD. We retrospectively reviewed the MDCT findings from a consecutive series of 25 patients diagnosed with PVOD, 9 with the sporadic form and 16 with the hereditary form of the disease. The presence and extent of typical findings of the diagnostic triad were assessed in all patients (ground glass parenchymal involvement, septal lines, and lymphadenopathy). In our series, 92% of patients showed at least two of the radiological findings described as typical of the disease. All patients presented at least one typical radiological characteristic. The incidence of radiological findings considered typical is very high, however was not associated with greater hemodynamic severity nor to the development of acute lung edema. No significant differences were found between the two groups. A poorly expressive MDCT does not exclude the disease. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

11 pages, 876 KiB  
Article
Soluble ST2 as a Biomarker for Early Complications in Patients with Chronic Thromboembolic Pulmonary Hypertension Treated with Balloon Pulmonary Angioplasty
by Marta Banaszkiewicz, Arkadiusz Pietrasik, Michał Florczyk, Piotr Kędzierski, Michał Piłka, Rafał Mańczak, Janusz Kochman, Grzegorz Opolski, Adam Torbicki, Marcin Kurzyna and Szymon Darocha
Diagnostics 2021, 11(1), 133; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics11010133 - 16 Jan 2021
Cited by 10 | Viewed by 2232
Abstract
Background: The aim of the study was to assess soluble ST2 (sST2) concentration and its dynamic changes in the periprocedural period in patients with chronic thromboembolic pulmonary hypertension (CTEPH) treated with balloon pulmonary angioplasty (BPA). Methods: We prospectively analyzed 57 procedures of BPA [...] Read more.
Background: The aim of the study was to assess soluble ST2 (sST2) concentration and its dynamic changes in the periprocedural period in patients with chronic thromboembolic pulmonary hypertension (CTEPH) treated with balloon pulmonary angioplasty (BPA). Methods: We prospectively analyzed 57 procedures of BPA performed in 37 patients with CTEPH. Biomarkers, such as N-terminal pro B-type natriuretic peptide (NT-proBNP), troponin T (TnT), and sST2 were assessed at four time points: Before the BPA procedure, 24 h and 48 h after the procedure, and at the discharge from hospital. Each postprocedural period was assessed for complications. Results: Before the BPA procedure, median sST2 concentration was 26.56 ng/mL (IQR: 16.66–40.83 ng/mL). sST2 concentration was significantly higher 24 h and 48 h after the BPA compared to the baseline measurements (33.31 ng/mL (IQR: 20.81–62.56), p = 0.000 and 27.45 ng/mL (IQR: 17.66–54.45), p = 0.028, respectively). sST2 level 24 h after the BPA procedure was significantly higher in the group with complications compared to the group without complications in the postprocedural period (97.66 ng/mL (IQR: 53.07–126.18) vs. 26.86 ng/mL (IQR: 19.10–40.12), p = 0.000). Conclusions: sST2 concentration in patients with CTEPH treated with BPA changes significantly in the postprocedural period and is significantly higher in the group with complications in postprocedural period. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

9 pages, 211 KiB  
Communication
COVID-19 in Pulmonary Artery Hypertension (PAH) Patients: Observations from a Large PAH Center in New York City
by Roxana Sulica, Frank Cefali, Caroline Motschwiller, Rebecca Fenton, Anabela Barroso and Daniel Sterman
Diagnostics 2021, 11(1), 128; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics11010128 - 15 Jan 2021
Cited by 22 | Viewed by 2983
Abstract
Information on outcomes of COVID-19 in pulmonary arterial hypertension (PAH) patients is limited to a few case series and surveys. Here, we describe our experience at a large Pulmonary Hypertension Center in New York City at the height of the pandemic. We performed [...] Read more.
Information on outcomes of COVID-19 in pulmonary arterial hypertension (PAH) patients is limited to a few case series and surveys. Here, we describe our experience at a large Pulmonary Hypertension Center in New York City at the height of the pandemic. We performed a retrospective chart review of eleven consecutive PAH patients who were diagnosed with SARS-CoV-2 infection. We analyzed demographics, PAH severity, risk factors for COVID-19, and COVID-19 severity and outcomes. We found in our sample that 63.6% of patients required intensive care, and there was a 45.45% overall mortality. Most patients had a known COVID-19 contact and mean duration of symptoms prior to presentation was 12 days. Only 4/11 (36%) patients presented to a center with pulmonary hypertension expertise, all of whom survived. Most patients had at least moderate pulmonary hypertension with an average REVEAL score of 7.81 despite double or triple PAH therapy. Our cases series underscores the gravity of SARS-CoV-2 infection in patients with PAH. It also suggests possible interventions to prevent unfavorable outcomes such as preserving social distancing, PAH management optimization, and early and preferential presentation to a center with specialized expertise in PAH. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
10 pages, 712 KiB  
Article
Galectin-3 Plasma Levels Are Associated with Risk Profiles in Pulmonary Arterial Hypertension
by Laura Scelsi, Stefano Ghio, Benedetta Matrone, Letizia Mannucci, Catherine Klersy, Serenella Valaperta, Annalisa Turco, Alessandra Greco, Giuseppe Derosa and Luigi Oltrona Visconti
Diagnostics 2020, 10(11), 857; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics10110857 - 22 Oct 2020
Cited by 5 | Viewed by 1846
Abstract
Galectin-3 is a circulating biomarker of fibrosis whose prognostic role in pulmonary arterial hypertension (PAH) has not been fully explored. We undertook a pilot study to evaluate the relationship between galectin-3 plasma levels and validated risk scores in PAH. The study included 70 [...] Read more.
Galectin-3 is a circulating biomarker of fibrosis whose prognostic role in pulmonary arterial hypertension (PAH) has not been fully explored. We undertook a pilot study to evaluate the relationship between galectin-3 plasma levels and validated risk scores in PAH. The study included 70 PAH patients admitted to a single referral center from June 2016 to June 2018. Patients were stratified according to the REVEAL 2.0 risk score, according to the parameters suggested by the European Society of Cardiology and European Respiratory Society (ESC/ERS) Guidelines, and according to a focused echocardiographic assessment of right heart performance. The association between galectin-3 levels and risk profiles was evaluated by generalized linear regression model with adjustment for etiology. Galectin-3 plasma levels increased linearly in the three risk strata based on the REVEAL 2.0 score (from 16.0 ± 5.7 in low-risk to 22.4 ± 6.3 in intermediate-risk and in 26.9 ± 7.7 ng/mL in high-risk patients (p for trend < 0.001). Galectin-3 levels were significantly lower in low-risk patients defined according to the prognostic parameters of ESC/ERS Guidelines (delta between low-risk and intermediate/high-risk = −9.3, 95% CI −12.8 to −5.8, p < 0.001, p < 0.001). Additionally, galectin-3 levels were lower in the low-risk profile defined on the basis of the echocardiographic evaluation of right heart performance (delta between low-risk and intermediate-/high-risk = −6.3, 95% CI −9.9 to −2.7, p = 0.001). Galectin-3 plasma levels are directly associated with several risk profiles in PAH patients. The prognostic role of this biomarker in PAH is worthwhile to be explored in larger prospective studies. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

11 pages, 2028 KiB  
Article
Feasibility of a Noninvasive Operability Assessment in Chronic Thromboembolic Pulmonary Hypertension under Real-World Practice
by Adriana Rodriguez Chaverri, Yolanda Revilla Ostolaza, Maria Jesus Lopez-Gude, María Teresa Velazquez, Ines Ponz de Antonio, Sergio Alonso Charterina, Agustin Albarran Gonzalez-Trevilla, Marta Perez Nunez, Jose Luis Perez Vela, Rafael Morales Ruiz, Juan F. Delgado Jimenez, Fernando Arribas Ynsaurriaga, Jose Maria Cortina and Pilar Escribano Subias
Diagnostics 2020, 10(10), 855; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics10100855 - 21 Oct 2020
Cited by 6 | Viewed by 1869
Abstract
This study aimed to evaluate the feasibility of a noninvasive operability assessment of chronic thromboembolic pulmonary hypertension (CTEPH) based on multidetector computed tomographic angiography (MCTA). Up to 176 patients were evaluated from January 2016 to April 2018. Throughout the first phase, the initial [...] Read more.
This study aimed to evaluate the feasibility of a noninvasive operability assessment of chronic thromboembolic pulmonary hypertension (CTEPH) based on multidetector computed tomographic angiography (MCTA). Up to 176 patients were evaluated from January 2016 to April 2018. Throughout the first phase, the initial surgical decision was made based on MCTA with further analysis of pulmonary angiography (PA) in order to evaluate in which cases the initial decision was not modified by PA. During the second phase, PA was limited to patients judged inoperable based on MCTA or those whose assessment was not possible. Patients deemed operable (50%) based on MCTA along the first phase had been adequately classified, as PA did not modify the initial decision in all but one patient. Comparable results were obtained throughout the implementation phase. Regarding operated patients, the decision of operability was based solely on MCTA in 94% of those with level I disease, in 75% with level II, and 54% with level III. This approach enabled shorter periods of time to complete surgical assessment and the avoidance of PA-related morbidity. Baseline parameters, postoperative measures, and survival rates at 1 year after surgery were comparable in both phases. Noninvasive operability assessment is feasible in a subset of CTEPH patients and optimizes surgical candidacy evaluation. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Graphical abstract

13 pages, 1116 KiB  
Article
Outcomes of Atrioseptostomy with Stenting in Patients with Pulmonary Arterial Hypertension from a Large Single-Institution Cohort
by Sergey V. Gorbachevsky, Anton A. Shmalts, Gulomjon M. Dadabaev, Nasirullo A. Nishonov, Manolis G. Pursanov, Vladimir A. Shvartz and Sergey B. Zaets
Diagnostics 2020, 10(9), 725; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics10090725 - 21 Sep 2020
Cited by 9 | Viewed by 2405
Abstract
The aim of this study was to analyze results of stenting atrioseptostomy in patients with pulmonary arterial hypertension and a different level of risk for one-year mortality that is not well described. Patients that underwent atrioseptostomy with stenting were retrospectively divided in two [...] Read more.
The aim of this study was to analyze results of stenting atrioseptostomy in patients with pulmonary arterial hypertension and a different level of risk for one-year mortality that is not well described. Patients that underwent atrioseptostomy with stenting were retrospectively divided in two groups: “intermediate” (n = 55) or “high” risk (n = 13), according to the 2015 ESC/ESR guideline. Results of atrioseptostomy were assessed during hospital period and at follow-up. Patients from “intermediate” risk group demonstrated lower mortality rate (10/55, vs. 6/13) during the course of the study period, as well as higher freedom from lung transplantation or Potts shunt. At discharge, patients of both groups presented improvement in functional class and mobility. Patients from “intermediate” risk group showed longer 6-min walking distance, and lower levels of brain natriuretic peptide. At the latest follow-up, stable position and full patency of stents with right-to-left or bidirectional shunt at atrial level and absence of syncope was confirmed in patients of both groups. Patients from the “intermediate” risk group demonstrated higher functional class, better performance of walking test, and lower levels of brain natriuretic peptide. Stenting atrioseptostomy reliably secured interatrial communication and improved clinical condition in patients with idiopathic pulmonary arterial hypertension. Mid-term results were better in “intermediate” risk group. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

15 pages, 2245 KiB  
Article
Prediction of Prognostic Hemodynamic Indices in Pulmonary Hypertension Using Non-Invasive Parameters
by Rafał Mańczak, Marcin Kurzyna, Michał Piłka, Szymon Darocha, Michał Florczyk, Maria Wieteska-Miłek, Małgorzata Mańczak and Adam Torbicki
Diagnostics 2020, 10(9), 644; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics10090644 - 27 Aug 2020
Cited by 4 | Viewed by 2622
Abstract
Effective targeted therapy of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) requires regular risk stratification. Among many prognostic parameters, three hemodynamic indices: right atrial pressure, cardiac index, and mixed venous saturation are considered critically important for correct risk classification. All [...] Read more.
Effective targeted therapy of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) requires regular risk stratification. Among many prognostic parameters, three hemodynamic indices: right atrial pressure, cardiac index, and mixed venous saturation are considered critically important for correct risk classification. All of them are measured invasively and require right heart catheterization (RHC). The study was aimed to verify assumption that a model based on non-invasive parameters is able to predict hemodynamic profile described by the mentioned invasive indices. A group of 330 patients with pulmonary hypertension was used for the selection of the best predictors from the set of 17 functional, biochemical, and echocardiographic parameters. Multivariable logistic regression models for the prediction of low-risk and high-risk profiles were created. The cut-off points were determined and subsequent validation of the models was conducted prospectively on another group of 136 patients. The ROC curve analysis showed the very good discrimination power of the models (AUC 0.80–0.99) in the prediction of the hemodynamic profile in the total validation group and subgroups: PAH and CTEPH. The models indicated the risk profiles with moderate sensitivity (57–60%) and high specificity (87–93%). The method enables estimation of the hemodynamic indices when RHC cannot be performed. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

Review

Jump to: Editorial, Research, Other

17 pages, 7887 KiB  
Review
Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact
by Mario Naranjo and Paul M. Hassoun
Diagnostics 2021, 11(5), 911; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics11050911 - 20 May 2021
Cited by 16 | Viewed by 4121
Abstract
Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a catastrophic complication of one of the most common and devastating autoimmune diseases. Once diagnosed, it becomes the leading cause of mortality among this patient population. Screening modalities and risk assessments have been designed and validated [...] Read more.
Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a catastrophic complication of one of the most common and devastating autoimmune diseases. Once diagnosed, it becomes the leading cause of mortality among this patient population. Screening modalities and risk assessments have been designed and validated by various organizations and societies in order to identify patients early in their disease course and promptly refer them to expert centers for a hemodynamic assessment and formal diagnosis. Moreover, several large multicenter clinical trials have now included patients with SSc-PAH to assess their response to therapy. Despite an improved understanding of the condition and significant advances in supportive and targeted therapy, outcomes have remained far from optimal. Therefore, rigorous phenotyping and search for novel therapies are desperately needed for this devastating condition. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

20 pages, 3152 KiB  
Review
Pulmonary Hypertension in Association with Lung Disease: Quantitative CT and Artificial Intelligence to the Rescue? State-of-the-Art Review
by Krit Dwivedi, Michael Sharkey, Robin Condliffe, Johanna M. Uthoff, Samer Alabed, Peter Metherall, Haiping Lu, Jim M. Wild, Eric A. Hoffman, Andrew J. Swift and David G. Kiely
Diagnostics 2021, 11(4), 679; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics11040679 - 09 Apr 2021
Cited by 15 | Viewed by 4960
Abstract
Accurate phenotyping of patients with pulmonary hypertension (PH) is an integral part of informing disease classification, treatment, and prognosis. The impact of lung disease on PH outcomes and response to treatment remains a challenging area with limited progress. Imaging with computed tomography (CT) [...] Read more.
Accurate phenotyping of patients with pulmonary hypertension (PH) is an integral part of informing disease classification, treatment, and prognosis. The impact of lung disease on PH outcomes and response to treatment remains a challenging area with limited progress. Imaging with computed tomography (CT) plays an important role in patients with suspected PH when assessing for parenchymal lung disease, however, current assessments are limited by their semi-qualitative nature. Quantitative chest-CT (QCT) allows numerical quantification of lung parenchymal disease beyond subjective visual assessment. This has facilitated advances in radiological assessment and clinical correlation of a range of lung diseases including emphysema, interstitial lung disease, and coronavirus disease 2019 (COVID-19). Artificial Intelligence approaches have the potential to facilitate rapid quantitative assessments. Benefits of cross-sectional imaging include ease and speed of scan acquisition, repeatability and the potential for novel insights beyond visual assessment alone. Potential clinical benefits include improved phenotyping and prediction of treatment response and survival. Artificial intelligence approaches also have the potential to aid more focused study of pulmonary arterial hypertension (PAH) therapies by identifying more homogeneous subgroups of patients with lung disease. This state-of-the-art review summarizes recent QCT developments and potential applications in patients with PH with a focus on lung disease. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

7 pages, 1316 KiB  
Review
Milestones in Understanding Pulmonary Circulation: From Antiquity to Heart-Lung Machine
by Adam Torbicki
Diagnostics 2021, 11(2), 381; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics11020381 - 23 Feb 2021
Cited by 1 | Viewed by 2430
Abstract
Human pulmonary circulation is as full of mysteries and surprises as is the history of attempts to uncover and understand them. The Special Issue of Diagnostics, appearing after 2020 immobilized the world, give us an opportunity, space and momentum to remind to [...] Read more.
Human pulmonary circulation is as full of mysteries and surprises as is the history of attempts to uncover and understand them. The Special Issue of Diagnostics, appearing after 2020 immobilized the world, give us an opportunity, space and momentum to remind to our medical community at least the main milestones which mark the progress that was made before our times. This review’s aim is to remind about pioneers and their ideas which now are considered as if they were always with us—which is not the case… Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

25 pages, 1841 KiB  
Review
Echocardiography for the Assessment of Pulmonary Hypertension and Congenital Heart Disease in the Young
by Katharina Meinel, Martin Koestenberger, Hannes Sallmon, Georg Hansmann and Guido E. Pieles
Diagnostics 2021, 11(1), 49; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics11010049 - 31 Dec 2020
Cited by 11 | Viewed by 6653
Abstract
While invasive assessment of hemodynamics and testing of acute vasoreactivity in the catheterization laboratory is the gold standard for diagnosing pulmonary hypertension (PH) and pulmonary vascular disease (PVD) in children, transthoracic echocardiography (TTE) serves as the initial diagnostic tool. International guidelines suggest several [...] Read more.
While invasive assessment of hemodynamics and testing of acute vasoreactivity in the catheterization laboratory is the gold standard for diagnosing pulmonary hypertension (PH) and pulmonary vascular disease (PVD) in children, transthoracic echocardiography (TTE) serves as the initial diagnostic tool. International guidelines suggest several key echocardiographic variables and indices for the screening studies when PH is suspected. However, due to the complex anatomy and special physiological considerations, these may not apply to patients with congenital heart disease (CHD). Misinterpretation of TTE variables can lead to delayed diagnosis and therapy, with fatal consequences, or–on the other hand-unnecessary invasive diagnostic procedures that have relevant risks, especially in the pediatric age group. We herein provide an overview of the echocardiographic workup of children and adolescents with PH with a special focus on children with CHD, such as ventricular/atrial septal defects, tetralogy of Fallot or univentricular physiology. In addition, we address the use of echocardiography as a tool to assess eligibility for exercise and sports, a major determinant of quality of life and outcome in patients with PH associated with CHD. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

11 pages, 888 KiB  
Review
Right Heart Size and Right Ventricular Reserve in Pulmonary Hypertension: Impact on Management and Prognosis
by Ekkehard Grünig, Christina A. Eichstaedt, Rebekka Seeger and Nicola Benjamin
Diagnostics 2020, 10(12), 1110; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics10121110 - 21 Dec 2020
Cited by 6 | Viewed by 5762
Abstract
Various parameters reflecting right heart size, right ventricular function and capacitance have been shown to be prognostically important in patients with pulmonary hypertension (PH). In the advanced disease, patients suffer from right heart failure, which is a main reason for an impaired prognosis. [...] Read more.
Various parameters reflecting right heart size, right ventricular function and capacitance have been shown to be prognostically important in patients with pulmonary hypertension (PH). In the advanced disease, patients suffer from right heart failure, which is a main reason for an impaired prognosis. Right heart size has shown to be associated with right ventricular function and reserve and is correlated with prognosis in patients with PH. Right ventricular reserve, defined as the ability of the ventricle to adjust to exercise or pharmacologic stress, is expressed by various parameters, which may be determined invasively by right heart catheterization or by stress-Doppler-echocardiography as a noninvasive approach. As the term “right ventricular contractile reserve” may be misleading, “right ventricular output reserve” seems desirable as a preferred term of increase in cardiac output during exercise. Both right heart size and right ventricular reserve have been shown to be of prognostic importance and may therefore be useful for risk assessment in patients with pulmonary hypertension. In this article we aim to display different aspects of right heart size and right ventricular reserve and their prognostic role in PH. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

27 pages, 6649 KiB  
Review
From Early Morphometrics to Machine Learning—What Future for Cardiovascular Imaging of the Pulmonary Circulation?
by Deepa Gopalan and J. Simon R. Gibbs
Diagnostics 2020, 10(12), 1004; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics10121004 - 25 Nov 2020
Cited by 3 | Viewed by 4537
Abstract
Imaging plays a cardinal role in the diagnosis and management of diseases of the pulmonary circulation. Behind the picture itself, every digital image contains a wealth of quantitative data, which are hardly analysed in current routine clinical practice and this is now being [...] Read more.
Imaging plays a cardinal role in the diagnosis and management of diseases of the pulmonary circulation. Behind the picture itself, every digital image contains a wealth of quantitative data, which are hardly analysed in current routine clinical practice and this is now being transformed by radiomics. Mathematical analyses of these data using novel techniques, such as vascular morphometry (including vascular tortuosity and vascular volumes), blood flow imaging (including quantitative lung perfusion and computational flow dynamics), and artificial intelligence, are opening a window on the complex pathophysiology and structure–function relationships of pulmonary vascular diseases. They have the potential to make dramatic alterations to how clinicians investigate the pulmonary circulation, with the consequences of more rapid diagnosis and a reduction in the need for invasive procedures in the future. Applied to multimodality imaging, they can provide new information to improve disease characterization and increase diagnostic accuracy. These new technologies may be used as sophisticated biomarkers for risk prediction modelling of prognosis and for optimising the long-term management of pulmonary circulatory diseases. These innovative techniques will require evaluation in clinical trials and may in themselves serve as successful surrogate end points in trials in the years to come. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

17 pages, 1134 KiB  
Review
Effect of Coronavirus Disease 2019 in Pulmonary Circulation. The Particular Scenario of Precapillary Pulmonary Hypertension
by Jorge Nuche, Teresa Segura de la Cal, Carmen Jiménez López Guarch, Francisco López-Medrano, Carmen Pérez-Olivares Delgado, Fernando Arribas Ynsaurriaga, Juan F. Delgado, Borja Ibáñez, Eduardo Oliver and Pilar Escribano Subías
Diagnostics 2020, 10(8), 548; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics10080548 - 31 Jul 2020
Cited by 21 | Viewed by 5784
Abstract
The Coronavirus Disease of 2019 (COVID-19) has supposed a global health emergency affecting millions of people, with particular severity in the elderly and patients with previous comorbidities, especially those with cardiovascular disease. Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension [...] Read more.
The Coronavirus Disease of 2019 (COVID-19) has supposed a global health emergency affecting millions of people, with particular severity in the elderly and patients with previous comorbidities, especially those with cardiovascular disease. Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) could represent an especially vulnerable population because of the high mortality rates reported for respiratory infections. However, the number of COVID-19 cases reported among PAH and CTEPH patients is surprisingly low. Furthermore, the clinical picture that has been described in these patients is far from the severity that experts would expect. Endothelial dysfunction is a common feature between patients with PAH/CTEPH and COVID-19, leading to ventilation/perfusion mismatch, vasoconstriction, thrombosis and inflammation. In this picture, the angiotensin-converting enzyme 2 plays an essential role, being directly involved in the pathophysiology of both clinical entities. Some of these common characteristics could explain the good adaptation of PAH and CTEPH patients to COVID-19, who could also have obtained a benefit from the disease’s specific treatments (anticoagulant and pulmonary vasodilators), probably due to its protective effect on the endothelium. Additionally, these common features could also lead to PAH/CTEPH as a potential sequelae of COVID-19. Throughout this comprehensive review, we describe the similarities and differences between both conditions and the possible pathophysiological and therapeutic-based mechanisms leading to the low incidence and severity of COVID-19 reported in PAH/CTEPH patients to date. Nevertheless, international registries should look carefully into this population for better understanding and management. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

Other

12 pages, 3187 KiB  
Perspective
The Challenge to Decide between Pulmonary Hypertension Due to Chronic Lung Disease and PAH with Chronic Lung Disease
by Horst Olschewski
Diagnostics 2021, 11(2), 311; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics11020311 - 15 Feb 2021
Cited by 13 | Viewed by 4331
Abstract
Chronic lung diseases are strongly associated with pulmonary hypertension (PH), and even mildly elevated pulmonary arterial pressures are associated with increased mortality. Chronic obstructive pulmonary disease (COPD) is the most common chronic lung disease, but few of these patients develop severe PH. Not [...] Read more.
Chronic lung diseases are strongly associated with pulmonary hypertension (PH), and even mildly elevated pulmonary arterial pressures are associated with increased mortality. Chronic obstructive pulmonary disease (COPD) is the most common chronic lung disease, but few of these patients develop severe PH. Not all these pulmonary pressure elevations are due to COPD, although patients with severe PH due to COPD may represent the largest subgroup within patients with COPD and severe PH. There are also patients with left heart disease (group 2), chronic thromboembolic disease (group 4, CTEPH) and pulmonary arterial hypertension (group 1, PAH) who suffer from COPD or another chronic lung disease as co-morbidity. Because therapeutic consequences very much depend on the cause of pulmonary hypertension, it is important to complete the diagnostic procedures and to decide on the main cause of PH before any decision on PAH drugs is made. The World Symposia on Pulmonary Hypertension (WSPH) have provided guidance for these important decisions. Group 2 PH or complex developmental diseases with elevated postcapillary pressures are relatively easy to identify by means of elevated pulmonary arterial wedge pressures. Group 4 PH can be identified or excluded by perfusion lung scans in combination with chest CT. Group 1 PAH and Group 3 PH, although having quite different disease profiles, may be difficult to discern sometimes. The sixth WSPH suggests that severe pulmonary hypertension in combination with mild impairment in the pulmonary function test (FEV1 > 60 and FVC > 60%), mild parenchymal abnormalities in the high-resolution CT of the chest, and circulatory limitation in the cardiopulmonary exercise test speak in favor of Group 1 PAH. These patients are candidates for PAH therapy. If the patient suffers from group 3 PH, the only possible indication for PAH therapy is severe pulmonary hypertension (mPAP ≥ 35 mmHg or mPAP between 25 and 35 mmHg together with very low cardiac index (CI) < 2.0 L/min/m2), which can only be derived invasively. Right heart catheter investigation has been established nearly 100 years ago, but there are many important details to consider when reading pulmonary pressures in spontaneously breathing patients with severe lung disease. It is important that such diagnostic procedures and the therapeutic decisions are made in expert centers for both pulmonary hypertension and chronic lung disease. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

2 pages, 167 KiB  
Comment
The Long and Winding Road of Atrial Septostomy
by Julio Sandoval
Diagnostics 2020, 10(11), 971; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics10110971 - 19 Nov 2020
Cited by 3 | Viewed by 1318
Abstract
Almost forty years have elapsed since the first description of the use of atrial septostomy in the setting of pulmonary arterial hypertension (PAH) [...] Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
6 pages, 1740 KiB  
Case Report
Balloon Pulmonary Angioplasty with Stent Implantation as a Treatment of Proximal Chronic Thromboembolic Pulmonary Hypertension
by Szymon Darocha, Radosław Pietura, Marta Banaszkiewicz, Arkadiusz Pietrasik, Łukasz Kownacki, Adam Torbicki and Marcin Kurzyna
Diagnostics 2020, 10(6), 363; https://0-doi-org.brum.beds.ac.uk/10.3390/diagnostics10060363 - 01 Jun 2020
Cited by 12 | Viewed by 2966
Abstract
We present a case of a 67-year-old female with proximal chronic thromboembolic pulmonary hypertension (CTEPH), disqualified from pulmonary endarterectomy due to multiple comorbidities and high risk-to-benefit ratio as assessed by multidisciplinary CTEPH team. She was referred for balloon pulmonary angioplasty (BPA) and underwent [...] Read more.
We present a case of a 67-year-old female with proximal chronic thromboembolic pulmonary hypertension (CTEPH), disqualified from pulmonary endarterectomy due to multiple comorbidities and high risk-to-benefit ratio as assessed by multidisciplinary CTEPH team. She was referred for balloon pulmonary angioplasty (BPA) and underwent three sessions with balloon catheters up to 8 mm diameter. During the second procedure, the elastic recoil phenomenon was observed in the treated post-thrombotic lesion of the right lower lobe artery, which made the balloon angioplasty ineffective. It was decided to implant a self-expanding stent for the prevention of restenosis. The procedure resulted in significant improvement of regional perfusion, as confirmed by control angiography. We feel that it contributed to the significant improvement of hemodynamic parameters and exercise capacity, as assessed three months after the last BPA procedure. In conclusion, pulmonary artery stenting may be an option in proximal CTEPH when elastic recoil phenomenon makes balloon angioplasty of a large vessel ineffective. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Diagnosis and Management)
►▼ Show Figures

Figure 1

Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:
 
Displaying articles 1-18
Back to TopTop