Pulmonary Hypertension: Symptoms, Diagnosis and Management

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Pulmonology".

Deadline for manuscript submissions: 20 December 2024 | Viewed by 9737

Special Issue Editors


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Guest Editor
Department of Pulmonology, Lithuanian University of Health Sciences, LT-44307 Kaunas, Lithuania
Interests: pulmonary vascular diseases; lung cancer

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Guest Editor
Department of Cardiology, Medical Academy, Lithuanian University of Health Sciences, LT-44307 Kaunas, Lithuania
Interests: heart left ventricle; pulmonary hypertension; echocardiography

Special Issue Information

Dear Colleagues,

This Special Issue is focused on the most recent data covering the various aspects of pulmonary hypertension, including clinical presentation, diagnosis confirmation, and management. Multiple etiologies cause elevated blood pressure in pulmonary arteries. Although several approved and recently introduced treatment possibilities exist, including the medications mostly for Group I (pulmonary arterial hypertension) and Group IV (pulmonary hypertension associated with pulmonary artery obstructions), the prognosis is poor. After the closure of small pulmonary arteries, the patients die due to the progressing failure of the right ventricle. There is an unmet need for a better understanding of this pathology for better management and patient care.

The aim and scope of this Special Issue are to help and encourage scientists working in this area to publish their data.

Prof. Dr. Skaidrius Miliauskas
Prof. Dr. Eglė Ereminienė
Guest Editors

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Keywords

  • pulmonary hypertension
  • cardiopulmonary exercise testing
  • lung function tests
  • echocardiography
  • cardiopulmonary exercise testing
  • lung function tests
  • cardiac magnetic resonance imaging

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Published Papers (6 papers)

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10 pages, 948 KiB  
Article
Prevalence, Predictors, and Outcomes of Pulmonary Hypertension in Patients with Lupus Nephritis
by Sixiu Chen, Junhan Guo, Xiamin Huang, Wei He, Xueqing Yu, Xi Xia and Wei Chen
Medicina 2024, 60(6), 988; https://doi.org/10.3390/medicina60060988 - 17 Jun 2024
Cited by 1 | Viewed by 1371
Abstract
Background and Objectives: This study aimed to assess the prevalence, predictors, and outcomes of pulmonary hypertension (PH) in patients with lupus nephritis (LN). Materials and Methods: Baseline characteristics and clinical outcomes of 387 patients with LN were retrospectively collected from 2007 to 2017. [...] Read more.
Background and Objectives: This study aimed to assess the prevalence, predictors, and outcomes of pulmonary hypertension (PH) in patients with lupus nephritis (LN). Materials and Methods: Baseline characteristics and clinical outcomes of 387 patients with LN were retrospectively collected from 2007 to 2017. PH was defined as pulmonary artery systolic pressure ≥40 mmHg assessed by resting transthoracic echocardiography. The primary endpoint was all-cause mortality. The secondary endpoint was renal events, defined as the doubling of baseline serum creatinine or end-stage renal disease. Associations between PH and outcomes were analyzed by Cox regression models. Results: A total of 15.3% (59/387) of patients with LN were diagnosed with PH, and the prevalence of PH was higher for patients with an estimated glomerular filtration rate (eGFR) < 30 mL/min/1.73 m2 compared to those with an eGFR ≥ 30 mL/min/1.73 m2 (31.5% vs. 12.6%). Higher mean arterial pressure, lower hemoglobin, and lower triglyceride levels were associated with greater odds of having PH. After adjusting for relevant confounding variables, PH was independently associated with a higher risk for death (HR: 2.01; 95% CI: 1.01–4.00; p = 0.047) and renal events (HR: 2.07; 95% CI: 1.04–4.12; p = 0.039). Conclusions: PH is an independent risk factor for all-cause mortality and adverse renal outcomes in patients with LN. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Symptoms, Diagnosis and Management)
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13 pages, 1360 KiB  
Article
The Impact of COVID-19 Infection on Patients with Severe Chronic Pulmonary Hypertension: A Prospective Study from a Single Referral Center
by Virginija Rudienė, Lina Kaplerienė, Monika Laukytė-Slėnienė, Dovilė Žebrauskienė, Vaida Averjanovaitė, Virginija Šileikienė, Ingrida Zeleckienė, Mindaugas Matačiūnas, Lina Gumbienė and Eglė Grigonienė
Medicina 2024, 60(5), 750; https://doi.org/10.3390/medicina60050750 - 30 Apr 2024
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Abstract
Background and Objectives. COVID-19 infection has a significant burden on global morbidity and mortality, especially in elderly people and in patients with chronic respiratory and cardiovascular diseases, such as pulmonary hypertension (PH). We aimed to evaluate the impact of COVID-19 infection on patients [...] Read more.
Background and Objectives. COVID-19 infection has a significant burden on global morbidity and mortality, especially in elderly people and in patients with chronic respiratory and cardiovascular diseases, such as pulmonary hypertension (PH). We aimed to evaluate the impact of COVID-19 infection on patients diagnosed with severe chronic PH. Materials and Methods. A single-center prospective cohort study was performed. Patients were enrolled from 1 November 2020 to 31 December 2022. Follow-up was until 31 December 2023. Data were collected on PH diagnosis, clinical presentation, outcomes, brain natriuretic peptide (BNP) levels, pulmonary function test with lung diffusion capacity for carbon monoxide (DLCO), and computed tomography pulmonary angiography (CTPA) analysis. Results. During the 26 months of our study, 51 PH patients were diagnosed with COVID-19 infection. The majority, 44 (86.3%) of all COVID-19 infected patients, were treated on an outpatient basis, and 7 (13.7%) required hospitalization. During the follow-up period, 8 (15.7%) patients died: 4 (7.8%) due to complications of COVID-19 infection, and the other 4 (7.8%) died in the later stages of the follow-up period after recovery from acute COVID-19 infection. Therefore, the in-hospital mortality in our study was 43% (n = 3). As mentioned above, the overall mortality was 7.8% (n = 4). Higher BNP levels in the third month after COVID-19 were associated with higher mortality rates (p = 0.028). Lung function, including DLCO, did not significantly worsen with COVID-19. In our study, 24 patients (47.1%) were referred for a follow-up CTPA scan and one of them developed typical fibrotic lung changes after COVID-19. Conclusions. The incidence of COVID-19 infection in patients diagnosed with PH was 34%. In our patients with severe chronic PH, the overall mortality rate due to COVID-19 infection was low. Pulmonary fibrosis was a rare complication in our cohort. COVID-19 infection in severe PH may increase the risk of worsening chronic heart failure. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Symptoms, Diagnosis and Management)
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11 pages, 650 KiB  
Article
The Prognostic Value of One-Year Changes in Biventricular Mechanics for Three-Year Survival in Patients with Precapillary Pulmonary Hypertension: A Cardiovascular Magnetic Resonance Feature Tracking Study
by Lina Padervinskienė, Joana Ažukaitė, Deimantė Hoppenot, Aušra Krivickienė, Paulius Šimkus, Irena Nedzelskienė, Skaidrius Miliauskas and Eglė Ereminienė
Medicina 2024, 60(1), 141; https://doi.org/10.3390/medicina60010141 - 12 Jan 2024
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Abstract
Background and Objectives: The management of patients with pulmonary hypertension (PH) poses a considerable challenge. While baseline cardiac magnetic resonance imaging (cMRI) indices are recognized for survival prognosis in PH, the prognostic value of one-year changes in biventricular mechanics, especially as assessed [...] Read more.
Background and Objectives: The management of patients with pulmonary hypertension (PH) poses a considerable challenge. While baseline cardiac magnetic resonance imaging (cMRI) indices are recognized for survival prognosis in PH, the prognostic value of one-year changes in biventricular mechanics, especially as assessed using feature tracking (FT) technology, remains underexplored. This study aims to assess the predictive value of one-year change in cMRI-derived biventricular function and mechanics parameters, along with N-terminal pro-brain natriuretic peptide (NT-proBNP) levels and six-minute walking test (6MWT) results for three-year mortality in precapillary PH patients. Materials and Methods: In this retrospective study, 36 patients diagnosed with precapillary pulmonary hypertension (mPAP 55.0 [46.3–70.5] mmHg, pulmonary capillary wedge pressure 10.0 [6.0–11.0] mmHg) were included. Baseline and one-year follow-up cMRI assessments, clinical data, and NT-proBNP levels were analyzed. FT technology was utilized to assess biventricular strain parameters. Patients were categorized into survival and non-survival groups based on three-year outcomes. Statistical analyses, including univariate logistic regression and Cox regression, were performed to identify predictive parameters. Results: The observed three-year survival rate was 83.3%. Baseline right ventricle (RV) ejection fraction (EF) was significantly higher in the survival group compared to non-survivors (41.0 [33.75–47.25]% vs. 28.0 [23.5–36.3]%, p = 0.044), and values of ≤32.5% were linked to a 20-fold increase in mortality risk. RV septum longitudinal strain (LS) and RV global LS exhibited significant improvement over a one-year period in the survival group compared to the non-survival group (−1.2 [−6.4–1.6]% vs. 4.9 [1.5–6.7]%, p = 0.038 and −3.1 [−9.1–2.6]% vs. 4.5 [−2.1–8.5]%, p = 0.048, respectively). Declines in RV septum LS by ≥2.95% and in RV GLS by ≥3.60% were associated with a 25-fold and 8-fold increase in mortality risk, respectively. Conclusions: The decrease in right ventricular septal and global longitudinal strain over a one-year period demonstrates a significant predictive value and an association with an increased three-year mortality risk in patients with precapillary PH. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Symptoms, Diagnosis and Management)
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14 pages, 636 KiB  
Brief Report
The Initial Experience of Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension in Latvia
by Anna Krigere, Verners Roberts Kalejs, Ricards Kaulins, Ainars Rudzitis, Liga Bondare, Matiss Sablinskis, Aivars Lejnieks, Dana Kigitovica, Marcin Kurzyna and Andris Skride
Medicina 2024, 60(4), 568; https://doi.org/10.3390/medicina60040568 - 30 Mar 2024
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Abstract
Background: Treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent pulmonary hypertension after pulmonary endarterectomy (PEA) include targeted medical therapy and balloon pulmonary angioplasty (BPA). BPA is an emerging treatment modality that has been reported to improve functional capacity, pulmonary [...] Read more.
Background: Treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent pulmonary hypertension after pulmonary endarterectomy (PEA) include targeted medical therapy and balloon pulmonary angioplasty (BPA). BPA is an emerging treatment modality that has been reported to improve functional capacity, pulmonary hemodynamics, and right ventricular function. Reports from expert centers are promising, but more data are needed to make the results more generalizable. Materials and Methods: We conducted a prospective analysis of nine consecutive CTEPH patients who underwent balloon pulmonary angioplasty (BPA) sessions at Pauls Stradins Clinical University Hospital in Riga, Latvia between 1 April 2022 and 1 July 2023. We assessed World Health Organization (WHO) functional class, 6 min walk distance (6MWD), blood oxygen saturation (SpO2), brain natriuretic peptide (BNP) level at baseline and 3 months after the first BPA session. For two patients on whom repeated BPA sessions were performed, we additionally assessed cardiac output (CO), pulmonary vascular resistance (PVR), and mean pulmonary artery pressure (mPAP). Results: A total of 12 BPA procedures for nine patients were performed; repeated BPA sessions were performed for two patients. Our results show a reduction in BNP levels and improvement in WHO functional class, 6MWD, and SpO2 after the first BPA session. Improvement in 6MWD was statistically significant. Additionally, an improvement in pulmonary hemodynamic parameters was observed. Conclusions: Our data show that BPA is an effective interventional treatment modality, improving both the pulmonary hemodynamics and functional status. Moreover, BPA is safe and excellently tolerated. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Symptoms, Diagnosis and Management)
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8 pages, 4331 KiB  
Case Report
Acquired Cardiovascular Diseases in Patients with Pulmonary Hypertension Due to Congenital Heart Disease: A Case Report
by Eglė Ereminienė, Mantvydas Stuoka, Rasa Ordienė, Jurgita Plisienė, Skaidrius Miliauskas and Eglė Tamulėnaitė
Medicina 2024, 60(2), 266; https://doi.org/10.3390/medicina60020266 - 3 Feb 2024
Viewed by 1334
Abstract
Background: Advances in the diagnosis and treatment of congenital heart diseases (CHDs) have resulted in improved survival rates for CHD patients. Up to 90% of individuals with mild CHD and 40% with complex CHD now reach the age of 60. Previous studies have [...] Read more.
Background: Advances in the diagnosis and treatment of congenital heart diseases (CHDs) have resulted in improved survival rates for CHD patients. Up to 90% of individuals with mild CHD and 40% with complex CHD now reach the age of 60. Previous studies have indicated an elevated risk of atherosclerotic cardiovascular disease (ASCVD) and associated risk factors, morbidity, and mortality in adults with congenital heart disease (ACHD). However, there were no comprehensive guidelines for the prevention and management of acquired cardiovascular diseases (CVDs) in ACHD populations until recently. Case presentation: A 55-year-old man with Eisenmenger syndrome and comorbidities (arterial hypertension, heart failure, dyslipidemia, hyperuricemia, and a history of pulmonary embolism (PE)) presented with progressive breathlessness. The electrocardiogram (ECG) revealed signs of right ventricle (RV) hypertrophy and overload, while echocardiography showed reduced RV function, RV overload, and severe pulmonary hypertension (PH) signs, and preserved left ventricle (LV) function. After ruling out a new PE episode, acute coronary syndrome (ACS) was diagnosed, and percutaneous intervention was performed within 24–48 h of admission. Conclusions: This case highlights the importance of increased awareness of acquired heart diseases in patients with pulmonary hypertension due to CHD. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Symptoms, Diagnosis and Management)
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9 pages, 295 KiB  
Brief Report
Baseline Clinical Characteristics and Incidence of Chronic Thromboembolic Pulmonary Hypertension Patients in Latvia, 2019–2020
by Ričards Kauliņš, Ainārs Rudzītis, Aivars Lejnieks, Dana Kigitoviča and Andris Skride
Medicina 2023, 59(8), 1426; https://doi.org/10.3390/medicina59081426 - 6 Aug 2023
Cited by 1 | Viewed by 1528
Abstract
Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and progressive condition; however, the true characteristics of CTEPH are still unknown, as notable regional variations exist in terms of patients’ age, baseline hemodynamic data, and management choices. This report aims to investigate [...] Read more.
Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and progressive condition; however, the true characteristics of CTEPH are still unknown, as notable regional variations exist in terms of patients’ age, baseline hemodynamic data, and management choices. This report aims to investigate the baseline clinical characteristics, incidence, and risk factors associated with CTEPH patients in Latvia from 2019 to 2020. Methods: The data were analyzed from a prospective, nationwide, Latvian pulmonary hypertension registry for incident CTEPH cases. The patients’ clinical characteristics were assessed at the time of diagnosis. Results: During the course of this study, a cohort of 13 patients with CTEPH were included for analysis. Among the enrolled CTEPH patients, most exhibited low exercise and functional capacity, with a median (±IQR) 6 min walk distance of 300.0 (±150.0) m. The median values (±IQR) for mean pulmonary artery pressure and pulmonary vascular resistance were 40.0 ± 13.0 mmHg and 7.35 ± 2.82 Wood units, respectively. The most common risk factors for CTEPH were a history of acute pulmonary embolism and a blood group other than O. Conclusions: The findings of this report revealed the characteristics of the Latvian CTEPH population, indicating that a significant proportion of patients are elderly individuals with multiple comorbidities. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Symptoms, Diagnosis and Management)
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