Journal of Clinical Medicine

Editorial

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4 pages, 190 KiB

Open AccessEditorial

Pulmonary Hypertension: Current Diagnosis, Approach and Treatment at the Dawn of the New European Guidelines

by Stylianos E. Orfanos and George Giannakoulas

J. Clin. Med. 2022, 11(19), 5804; https://doi.org/10.3390/jcm11195804 - 30 Sep 2022

Cited by 2 | Viewed by 1572

Abstract

A turning point in the field of pulmonary hypertension (PH) is the most recent publication of the new European Guidelines for the diagnosis and treatment of pulmonary hypertension, a collaboration between the European Society of Cardiology and the European Respiratory Society [...] Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

Research

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14 pages, 1955 KiB

Open AccessArticle

Balloon Pulmonary Angioplasty in Patients with Chronic Thromboembolic Pulmonary Hypertension in Greece: Data from the Hellenic Pulmonary Hypertension Registry

by Panagiotis Karyofyllis, Eftychia Demerouti, George Giannakoulas, Anastasia Anthi, Alexandra Arvanitaki, George Athanassopoulos, Christos Feloukidis, Ioannis Iakovou, Theodora Kostelidou, Ioanna Mitrouska, Sophia-Anastasia Mouratoglou, Stylianos E. Orfanos, Christos Pappas, Georgia Pitsiou, Eleftheria-Garyfallia Tsetika, Dimitrios Tsiapras, Vassilios Voudris and Athanassios Manginas

J. Clin. Med. 2022, 11(8), 2211; https://doi.org/10.3390/jcm11082211 - 15 Apr 2022

Cited by 7 | Viewed by 2285

Abstract

Balloon pulmonary angioplasty (BPA) is a novel and promising treatment option for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy (PEA) and for those with persistent or recurrent pulmonary hypertension after PEA. We present the results of BPA [...] Read more.

Balloon pulmonary angioplasty (BPA) is a novel and promising treatment option for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy (PEA) and for those with persistent or recurrent pulmonary hypertension after PEA. We present the results of BPA procedures in CTEPH patients included in the Greek Pulmonary Hypertension Registry, evaluating the real-life efficacy and safety. We analyzed data from 180 BPA procedures (2–17/patient, mean 8 ± 4/patient, 1248 dilated vessels, 0–18/session). Significant improvements were observed in mean pulmonary arterial pressure (a reduction by 44%, p < 0.001), pulmonary vascular resistance (reduction by 60%, p < 0.001), and NT-proBNP (decrease by >70%, p: 0.003), while cardiac index improved modestly (9% increase, p = 0.143). We had 37 BPA-related non-fatal complications (20.6% in all interventions), predominantly including hemoptysis. Overall survival was 91%, 75% and 62% at 3, 4 and 5 years, respectively. Therefore, BPA may be a promising therapeutic option in patients with CTEPH in Greece. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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13 pages, 862 KiB

Open AccessArticle

Non-Invasive Risk Prediction Based on Right Ventricular Function in Patients with Pulmonary Arterial Hypertension

by Vazhma Qaderi, Jessica Weimann, Lars Harbaum, Benedikt N. Schrage, Dorit Knappe, Jan K. Hennigs, Christoph Sinning, Renate B. Schnabel, Stefan Blankenberg, Paulus Kirchhof, Hans Klose and Christina Magnussen

J. Clin. Med. 2021, 10(21), 5130; https://doi.org/10.3390/jcm10215130 - 31 Oct 2021

Cited by 2 | Viewed by 2158

Abstract

Background: Right ventricular dysfunction is a major determinant of outcome in pulmonary arterial hypertension (PAH). We aimed to identify echocardiographic right heart parameters associated with adverse outcome and to develop a non-invasive, echocardiography-based risk score for PAH patients. Methods and Results: In 254 [...] Read more.

Background: Right ventricular dysfunction is a major determinant of outcome in pulmonary arterial hypertension (PAH). We aimed to identify echocardiographic right heart parameters associated with adverse outcome and to develop a non-invasive, echocardiography-based risk score for PAH patients. Methods and Results: In 254 PAH patients we analyzed functional status, laboratory results, and echocardiographic parameters. We included these parameters to estimate all-cause death or lung transplantation using Cox regression models. The analyses included a conventional model using guideline-recommended variables and an extended echocardiographic model. Based on the final model a 12-point risk score was derived, indicating the association with the primary outcome within five years. During a median follow-up time of 4.2 years 74 patients died or underwent lung transplantation. The conventional model resulted in a C-Index of 0.539, whereas the extended echocardiographic model improved the discrimination (C-index 0.639, p-value 0.017). Ultimately, the newly developed risk score included WHO functional class, 6-min walking distance, N-terminal brain natriuretic peptide concentrations, pericardial effusion, right atrial area, tricuspid annular plane systolic excursion, and fractional area change. Conclusion: Integrating right heart function assessed by echocardiography improves prediction of death or lung transplantation in PAH patients. Independent validation of this finding is warranted. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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11 pages, 520 KiB

Open AccessArticle

Epidemiology and Management of Chronic Thromboembolic Pulmonary Hypertension in Greece. Real-World Data from the Hellenic Pulmonary Hypertension Registry (HOPE)

by Eftychia Demerouti, Panagiotis Karyofyllis, Vassilios Voudris, Maria Boutsikou, George Anastasiadis, Anastasia Anthi, Alexandra Arvanitaki, George Athanassopoulos, Aikaterini Avgeropoulou, Styliani Brili, Christos Feloukidis, Frantzeska Frantzeskaki, George Karatasakis, Haralambos Karvounis, Dimitrios Konstantonis, Ioanna Mitrouska, Sophia Mouratoglou, Katerina K. Naka, Stylianos E. Orfanos, Evangelia Panagiotidou, Georgia Pitsiou, Antonios Pitsis, Vagia Stamatopoulou, Ioannis Stanopoulos, Adina Thomaidis, Iraklis Tsangaris, Dimitrios Tsiapras, George Giannakoulas, Athanassios Manginas and on behalf of the Hellenic Society for the Study of Pulmonary Hypertension (HSSPH) Show full author list Hide full author list

J. Clin. Med. 2021, 10(19), 4547; https://doi.org/10.3390/jcm10194547 - 30 Sep 2021

Cited by 10 | Viewed by 2697

Abstract

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare disease with poor prognosis if left untreated, characterized by pulmonary vascular bed obstruction due to unresolving thromboembolic material. The Hellenic pulmonary hypertension registry (HOPE) was launched in Greece in early 2015 and enrolls patients from [...] Read more.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare disease with poor prognosis if left untreated, characterized by pulmonary vascular bed obstruction due to unresolving thromboembolic material. The Hellenic pulmonary hypertension registry (HOPE) was launched in Greece in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. In total, 98 patients with CTEPH were enrolled from January 2015 until November 2019. Of these patients, 55.1% represented incident population, 50% were classified in the World Health Organization functional class II and 49% had a history of acute pulmonary embolism. The median values of pulmonary vascular resistance (PVR) and cardiac index were 7.4 (4.8) WU and 2.4 (1.0) L/min/m², respectively, the mean diffusing capacity for carbon monoxide was 74.8 ± 20.6%, the median 6-minute walk distance was 347 (220) meters and the median value of N Terminal-pro brain natriuretic peptide was 506.0 (1450.0) pg/mL. In total, 60.2% of the patients were under pulmonary arterial hypertension-targeted therapy at the time of enrolment; specifically, riociguat was received by 35.7% of the patients and combination therapy was the preferred strategy for 16% of the patients. In total, 74 patients were evaluated for pulmonary endarterectomy (PEA), 34 (45.9%) were assessed as operable but only 23 of those (31.1%) finally underwent PEA. The remaining 40 patients were ineligible for PEA according to the operability assessment and 13 (17.6%) of them underwent balloon pulmonary angioplasty. The age of the non-operable patients was significantly higher than the operable patients (p < 0.001), while there was no significant difference with regard to the history of coagulopathies between the operable and non-operable patients (p = 0.33). Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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14 pages, 5335 KiB

Open AccessArticle

Sex Differences in Chronic Thromboembolic Pulmonary Hypertension. Treatment Options over Time in a National Referral Center

by Alejandro Cruz-Utrilla, María José Cristo-Ropero, Miguel Calderón-Flores, Maite Velázquez, María Jesús López-Gude, Yolanda Revilla Ostolaza, José Luis Pérez Vela, Javier de la Cruz-Bértolo, Héctor Bueno, Fernando Arribas Ynsaurriaga, José María Cortina and Pilar Escribano-Subias

J. Clin. Med. 2021, 10(18), 4251; https://doi.org/10.3390/jcm10184251 - 19 Sep 2021

Cited by 8 | Viewed by 2643

Abstract

(1) Background: Clinical presentation, disease distribution, or treatment received may provide insights into the reasons contributing to sex differences in chronic thromboembolic pulmonary hypertension (CTEPH). (2) Methods: We evaluated 453 patients (56% women) between 2007–2019. Data was collected from REHAP (Registro Español de [...] Read more.

(1) Background: Clinical presentation, disease distribution, or treatment received may provide insights into the reasons contributing to sex differences in chronic thromboembolic pulmonary hypertension (CTEPH). (2) Methods: We evaluated 453 patients (56% women) between 2007–2019. Data was collected from REHAP (Registro Español de Hipertensión Arterial Pulmonar) registry. Two time periods were selected to evaluate the influence of new treatments over time. (3) Results: Women were older. Baseline functional class was worse, and distance walked shorter in women compared with men. Women had higher pulmonary vascular resistances. Despite this, pulmonary endarterectomy (PEA) was carried out in more men, and women received more frequently pulmonary vasodilators exclusively. The 2014–2019 interval was associated with a better survival only among women. Interestingly, women had a more distal disease during this second period of time. (4) Conclusions: Even though women were older, and received invasive treatments less frequently, mortality was similar in both sexes. The introduction of balloon pulmonary angioplasty and the improvement of pulmonary endarterectomy, especially during the last years, could be associated with a survival benefit among women. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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10 pages, 390 KiB

Open AccessArticle

Low-Dose Dobutamine Stress Echocardiography for the Early Detection of Pulmonary Arterial Hypertension in Selected Patients with Systemic Sclerosis Whose Resting Echocardiography Is Non-Diagnostic for Pulmonary Hypertension

by Loukianos S. Rallidis, Konstantina Papangelopoulou, Georgios Makavos, Christos Varounis, Anastasia Anthi and Stylianos E. Orfanos

J. Clin. Med. 2021, 10(17), 3972; https://doi.org/10.3390/jcm10173972 - 2 Sep 2021

Cited by 2 | Viewed by 2006

Abstract

Background: Dobutamine stress echocardiography (DSE) has limited application in systemic sclerosis (SSc). We examined DSE usefulness in revealing pulmonary arterial hypertension (PAH) in selected SSc patients whose resting echocardiography for pulmonary hypertension (PH) was non-diagnostic. Methods: Forty SSc patients underwent right heart catheterization [...] Read more.

Background: Dobutamine stress echocardiography (DSE) has limited application in systemic sclerosis (SSc). We examined DSE usefulness in revealing pulmonary arterial hypertension (PAH) in selected SSc patients whose resting echocardiography for pulmonary hypertension (PH) was non-diagnostic. Methods: Forty SSc patients underwent right heart catheterization (RHC) and, simultaneously, low-dose DSE (incremental doses up to 20 μg/kg/min). Inclusion criteria were: preserved left and right ventricular (RV) function (tricuspid annulus plane systolic excursion [TAPSE] ≥ 16 mm and tissue Doppler imaging-derived systolic velocity of tricuspid annulus [RVS’] > 10 cm/s), normal pulmonary function tests, and baseline maximal tricuspid regurgitation (TR) velocity of 2.7–3.2 m/s. Results: Of 36 patients who completed DSE, resting RHC diagnosed PAH in 12 patients (33.3%). At 20 μg/kg/min, patients with PAH had higher TR velocity, higher pulmonary arterial pressure measured by RHC, and lower RV inotropic response compared with patients without PAH. A cut-off value of maximal TR velocity >3.1 m/s had a sensitivity of 80%, a specificity of 84.2%, and an accuracy of 82.4% for the detection of PAH. Conclusions: Low-dose DSE has a satisfactory diagnostic accuracy for the early detection of PAH in highly selected SSc patients whose baseline echocardiographic measurements for PH lie in the gray zone. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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16 pages, 776 KiB

Open AccessArticle

Nitric Oxide Stroke Volume Index as a New Hemodynamic Prognostic Parameter for Patients with Pulmonary Arterial Hypertension

by Karolina Barańska-Pawełczak, Celina Wojciechowska, Mariusz Opara and Wojciech Jacheć

J. Clin. Med. 2020, 9(9), 2939; https://doi.org/10.3390/jcm9092939 - 11 Sep 2020

Cited by 3 | Viewed by 2096

Abstract

The aim of the study was to determine the prognostic value of hemodynamic parameters measured during initial diagnostic right heart catheterization (RHC) in standard conditions and using a nitric oxide reversibility test. A retrospective observational study of 62 patients with pulmonary arterial hypertension [...] Read more.

The aim of the study was to determine the prognostic value of hemodynamic parameters measured during initial diagnostic right heart catheterization (RHC) in standard conditions and using a nitric oxide reversibility test. A retrospective observational study of 62 patients with pulmonary arterial hypertension (PAH) was performed. Clinical, biochemical, echocardiographic, and hemodynamic data obtained at the time of the PAH diagnosis were precisely analyzed. Patients were followed for five years. Death or lung transplantation was considered as a primary endpoint. The mean follow-up period was 1090 ± 703 days and the median age was 46.84 years. In the studied group, 25 patients survived, 36 patients died, and one underwent a lung transplantation. From all the examined parameters, only stroke volume index during reversibility test with iNO (SVI_{(NO test)}) (HR = 0.910; 95% confidence interval 0.878–0.944; p < 0.001) and initial arterial oxygen saturation (SaO₂) (HR = 0.910; 95% confidence interval 0.843–0.982; p = 0.015) have been established as independent predictors of death or lung transplantation in the five-year follow–up. An SVI_{(NO test)} value above 39.86 mL/m² was associated with 100% five-year survival rate (AUC = 0.956; 95% confidence interval 0.899–1.000; p < 0.001; specificity/sensitivity: 100/84%). The results of the analysis suggest that the SVI_{(NO test)} measured during the initial diagnostic RHC could be a very valuable prognostic factor in the PAH patients. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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Review

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12 pages, 884 KiB

Open AccessReview

The Role of Thyroid Disorders, Obesity, Diabetes Mellitus and Estrogen Exposure as Potential Modifiers for Pulmonary Hypertension

by Eleni Vrigkou, Evangeline Vassilatou, Effrosyni Dima, David Langleben, Anastasia Kotanidou and Marinella Tzanela

J. Clin. Med. 2022, 11(4), 921; https://doi.org/10.3390/jcm11040921 - 10 Feb 2022

Cited by 10 | Viewed by 3226

Abstract

Pulmonary hypertension (PH) is a progressive disorder characterized by a chronic in-crease in pulmonary arterial pressure, frequently resulting in right-sided heart failure and potentially death. Co-existing medical conditions are important factors in PH, since they not only result in the genesis of the [...] Read more.

Pulmonary hypertension (PH) is a progressive disorder characterized by a chronic in-crease in pulmonary arterial pressure, frequently resulting in right-sided heart failure and potentially death. Co-existing medical conditions are important factors in PH, since they not only result in the genesis of the disorder, but may also contribute to its progression. Various studies have assessed the impact of thyroid disorders and other endocrine conditions (namely estrogen exposure, obesity, and diabetes mellitus) on the progression of PH. The complex interactions that hormones may have with the cardiovascular system and pulmonary vascular bed can create several pathogenetic routes that could explain the effects of endocrine disorders on PH development and evolution. The aim of this review is to summarize current knowledge on the role of concomitant thyroid disorders, obesity, diabetes mellitus, and estrogen exposure as potential modifiers for PH, and especially for pulmonary arterial hypertension, and to discuss possible pathogenetic routes linking them with PH. This information could be valuable for practicing clinicians so as to better evaluate and/or treat concomitant endocrine conditions in the PH population. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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15 pages, 2364 KiB

Open AccessReview

Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management

by Amalia Baroutidou, Alexandra Arvanitaki, Adam Hatzidakis, Georgia Pitsiou, Antonios Ziakas, Haralambos Karvounis and George Giannakoulas

J. Clin. Med. 2022, 11(3), 633; https://doi.org/10.3390/jcm11030633 - 26 Jan 2022

Cited by 9 | Viewed by 10052

Abstract

Haemoptysis represents one of the most severe major bleeding manifestations in the clinical course of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Accumulating evidence indicates that dysfunction of the pulmonary vascular bed in the setting of PAH predisposes patients to [...] Read more.

Haemoptysis represents one of the most severe major bleeding manifestations in the clinical course of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Accumulating evidence indicates that dysfunction of the pulmonary vascular bed in the setting of PAH predisposes patients to increased hemorrhagic diathesis, resulting in mild to massive and life-threatening episodes of haemoptysis. Despite major advances in PAH targeted treatment strategies, haemoptysis is still correlated with substantial morbidity and impaired quality of life, requiring a multidisciplinary approach by adult CHD experts in tertiary centres. Technological innovations in the field of diagnostic and interventional radiology enabled the application of bronchial artery embolization (BAE), a valuable tool to efficiently control haemoptysis in modern clinical practice. However, bleeding recurrences are still prevalent, implying that the optimum management of haemoptysis and its implications remain obscure. Moreover, regarding the use of oral anticoagulation in patients with haemoptysis, current guidelines do not provide a clear therapeutic strategy due to the lack of evidence. This review aims to discuss the main pathophysiological mechanisms of haemoptysis in PAH-CHD, present the clinical spectrum and the available diagnostic tools, summarize current therapeutic challenges, and propose directions for future research in this group of patients. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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15 pages, 69950 KiB

Open AccessReview

Echocardiography in Pulmonary Arterial Hypertension: Comprehensive Evaluation and Technical Considerations

by Weronika Topyła-Putowska, Michał Tomaszewski, Andrzej Wysokiński and Andrzej Tomaszewski

J. Clin. Med. 2021, 10(15), 3229; https://doi.org/10.3390/jcm10153229 - 22 Jul 2021

Cited by 22 | Viewed by 8568

Abstract

Pulmonary arterial hypertension (PAH) is a rare, progressive disease in which there is a persistent, abnormal increase in pulmonary artery pressure. Symptoms of pulmonary hypertension are nonspecific and mainly associated with progressive right ventricular failure. The diagnosis of PAH is a multistep process [...] Read more.

Pulmonary arterial hypertension (PAH) is a rare, progressive disease in which there is a persistent, abnormal increase in pulmonary artery pressure. Symptoms of pulmonary hypertension are nonspecific and mainly associated with progressive right ventricular failure. The diagnosis of PAH is a multistep process and often requires the skillful use of several tests. The gold standard for the diagnosis of PAH is hemodynamic testing. Echocardiography currently plays an important role in the diagnostic algorithm of PAH as it is minimally invasive and readily available. Moreover, many echocardiographic parameters are closely related to pulmonary hemodynamics. It allows assessment of the right heart′s structure and function, estimation of the pressure in the right ventricle, right atrium, and pulmonary trunk, and exclusion of other causes of elevated pulmonary bed pressure. Echocardiographic techniques are constantly evolving, and recently, measurements made using new techniques, especially 3D visualization, have become increasingly important. In echocardiographic assessment, it is crucial to know current guidelines and new reports that organize the methodology and allow standardization of the examination. This review aims to discuss the different echocardiographic techniques used to evaluate patients with PAH. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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17 pages, 707 KiB

Open AccessReview

Echocardiography in Pulmonary Arterial Hypertension: Is It Time to Reconsider Its Prognostic Utility?

by Ioannis T. Farmakis, Eftychia Demerouti, Panagiotis Karyofyllis, George Karatasakis, Maria Stratinaki, Dimitrios Tsiapras, George Athanassopoulos, Vassilios Voudris and George Giannakoulas

J. Clin. Med. 2021, 10(13), 2826; https://doi.org/10.3390/jcm10132826 - 26 Jun 2021

Cited by 13 | Viewed by 11679

Abstract

Pulmonary arterial hypertension (PAH) is characterized by an insult in the pulmonary vasculature, with subsequent right ventricular (RV) adaptation to the increased afterload that ultimately leads to RV failure. The awareness of the importance of RV function in PAH has increased considerably because [...] Read more.

Pulmonary arterial hypertension (PAH) is characterized by an insult in the pulmonary vasculature, with subsequent right ventricular (RV) adaptation to the increased afterload that ultimately leads to RV failure. The awareness of the importance of RV function in PAH has increased considerably because right heart failure is the predominant cause of death in PAH patients. Given its wide availability and reduced cost, echocardiography is of paramount importance in the evaluation of the right heart in PAH. Several echocardiographic parameters have been shown to have prognostic implications in PAH; however, the role of echocardiography in the risk assessment of the PAH patient is limited under the current guidelines. This review discusses the echocardiographic evaluation of the RV in PAH and during therapy, and its prognostic implications, as well as the potential significant role of repeated echocardiographic assessment in the follow-up of patients with PAH. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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13 pages, 1351 KiB

Open AccessReview

Nailfold Capillaroscopy in Systemic Sclerosis Patients with and without Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis

by Ioanna Minopoulou, Marieta Theodorakopoulou, Afroditi Boutou, Alexandra Arvanitaki, Georgia Pitsiou, Michael Doumas, Pantelis Sarafidis and Theodoros Dimitroulas

J. Clin. Med. 2021, 10(7), 1528; https://doi.org/10.3390/jcm10071528 - 6 Apr 2021

Cited by 20 | Viewed by 3178

Abstract

Systemic sclerosis (SSc)-related pulmonary arterial hypertension (SSc-PAH) is a leading cause of mortality in SSc. The extent of peripheral microvasculopathy assessed through nailfold capillaroscopy might correlate with the presence of PAH in SSc patients. We searched the PubMed, Cochrane Library, Scopus, and Web [...] Read more.

Systemic sclerosis (SSc)-related pulmonary arterial hypertension (SSc-PAH) is a leading cause of mortality in SSc. The extent of peripheral microvasculopathy assessed through nailfold capillaroscopy might correlate with the presence of PAH in SSc patients. We searched the PubMed, Cochrane Library, Scopus, and Web of Science databases and performed a random effects meta-analysis of observational studies comparing nailfold capillaroscopic alterations in SSc-PAH versus SSc-noPAH patients. Weighted mean differences (WMD) with the corresponding confidence intervals (CIs) were estimated. The quality of the included studies was evaluated using a modified Newcastle–Ottawa scale. Seven studies with 101 SSc-PAH and 277 SSc-noPAH participants were included. Capillary density was marginally reduced in the SSc-PAH group (WMD: −1.0, 95% CI: −2.0 to 0.0, I² = 86%). This effect was strengthened once PAH diagnosis was confirmed by right heart catheterization (WMD: −1.2, 95% CI: −2.3 to −0.1, I² = 85%). An increase in capillary loop width was observed in SSc-PAH compared to SSc-noPAH patients (WMD: 10.9, 95% CI: 2.5 to 19.4, I² = 78%). Furthermore, SSc-PAH patients had a 7.3 times higher likelihood of active or late scleroderma pattern (95% CI: 3.0 to 18.0, I² = 4%). SSc-PAH patients presented with worse nailfold capillaroscopic findings compared to SSc-noPAH patients. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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Other

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9 pages, 1609 KiB

Open AccessCommentary

The Paradox of Pulmonary Vascular Resistance: Restoration of Pulmonary Capillary Recruitment as a Sine Qua Non for True Therapeutic Success in Pulmonary Arterial Hypertension

by David Langleben, Stylianos E. Orfanos, Benjamin D. Fox, Nathan Messas, Michele Giovinazzo and John D. Catravas

J. Clin. Med. 2022, 11(15), 4568; https://doi.org/10.3390/jcm11154568 - 5 Aug 2022

Cited by 5 | Viewed by 2174

Abstract

Exercise-induced increases in pulmonary blood flow normally increase pulmonary arterial pressure only minimally, largely due to a reserve of pulmonary capillaries that are available for recruitment to carry the flow. In pulmonary arterial hypertension, due to precapillary arteriolar obstruction, such recruitment is greatly [...] Read more.

Exercise-induced increases in pulmonary blood flow normally increase pulmonary arterial pressure only minimally, largely due to a reserve of pulmonary capillaries that are available for recruitment to carry the flow. In pulmonary arterial hypertension, due to precapillary arteriolar obstruction, such recruitment is greatly reduced. In exercising pulmonary arterial hypertension patients, pulmonary arterial pressure remains high and may even increase further. Current pulmonary arterial hypertension therapies, acting principally as vasodilators, decrease calculated pulmonary vascular resistance by increasing pulmonary blood flow but have a minimal effect in lowering pulmonary arterial pressure and do not restore significant capillary recruitment. Novel pulmonary arterial hypertension therapies that have mainly antiproliferative properties are being developed to try and diminish proliferative cellular obstruction in precapillary arterioles. If effective, those agents should restore capillary recruitment and, during exercise testing, pulmonary arterial pressure should remain low despite increasing pulmonary blood flow. The effectiveness of every novel therapy for pulmonary arterial hypertension should be evaluated not only at rest, but with measurement of exercise pulmonary hemodynamics during clinical trials. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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3 pages, 197 KiB

Open AccessComment

Comment on Topyła-Putowska et al. Echocardiography in Pulmonary Arterial Hypertension: Comprehensive Evaluation and Technical Considerations. J. Clin. Med. 2021, 10, 3229

by Giovanna Manzi, Carmine Dario Vizza and Roberto Badagliacca

J. Clin. Med. 2022, 11(12), 3337; https://doi.org/10.3390/jcm11123337 - 10 Jun 2022

Cited by 2 | Viewed by 1124

Abstract

A comprehensive PAH echocardiographic examination of patients with pulmonary arterial hypertension (PAH) should include a set of parameters resembling the pathophysiological changes occurring in the course of the disease. This approach could help clinicians build a complete picture of the patient, test the [...] Read more.

A comprehensive PAH echocardiographic examination of patients with pulmonary arterial hypertension (PAH) should include a set of parameters resembling the pathophysiological changes occurring in the course of the disease. This approach could help clinicians build a complete picture of the patient, test the effects of targeted therapies and identify patients who need a more aggressive therapeutic approach to achieve a low risk-status. Full article

(This article belongs to the Special Issue Pulmonary Hypertension: Current Diagnosis Approach and Treatment)

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