Pulmonary Hypertension: Updates in Diagnosis and Management

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

Deadline for manuscript submissions: closed (20 October 2023) | Viewed by 15631

Special Issue Editors


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Guest Editor
Pulmonary Hypertension, Right Heart Failure, CTEPH Program, Temple Heart and Vascular Institute, Temple University Hospital, Philadelphia, PA 19140, USA
Interests: pulmonary hypertension; right heart failure; chronic thromboembolic pulmonary hypertension (CTEPH); pulmonary thromboendarterectomy (PTE); advanced heart failure cardiac transplant; ventricular assist device (VAD); cardiopulmonary exercise testing (CPET); right heart catheterization; exercise physiology; echocardiography; pulmonary embolism venous thromboembolism

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Guest Editor
Division of Cardiology, Temple University Hospital, Philadelphia, PA 19140, USA
Interests: heart failure; pulmonary hypertension; chronic thromboembolic pulmonary hypertension (CTEPH)

E-Mail Website
Guest Editor
Pulmonary Hypertension, Right Heart Failure, CTEPH Program, Temple Heart and Vascular Institute, Temple University Hospital, Philadelphia, PA 19140, USA
Interests: pulmonary hypertension; chronic thromboembolic pulmonary hypertension (CTEPH)

Special Issue Information

Dear Colleagues,

Pulmonary hypertension is a major global health issue estimated to affect 1% of the global population and is thought to be associated with multiple cardiovascular and respiratory diseases. Irrespective of the underlying condition, PH is associated with worsening symptoms and increased mortality. However, in the last decade, substantial progress has been made in the diagnosis and management of pulmonary hypertension.

This Special Issue (SI) of the Journal of Clinical Medicine, entitled “Pulmonary Hypertension: Updates in Diagnosis and Management”, aims to publish review articles and original research related to recent advances in surgical as well as pharmacological therapy for pulmonary hypertension. We will include ground-breaking research articles of significant clinical and scientific value, including observational studies, review articles and clinical trials.

Prof. Dr. Anjali Vaidya
Dr. Anil Kumar Jonnalagadda
Dr. Estefanía Oliveros
Guest Editors

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Keywords

  • pulmonary hypertension
  • heart failure
  • surgery
  • drugs
  • COVID-19
  • advances

Published Papers (8 papers)

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Research

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12 pages, 809 KiB  
Article
Intravenous Diuresis in Severe Precapillary Pulmonary-Hypertension-Related Right Heart Failure: Effects on Renal Function and Blood Pressure
by Lyana Labrada, Carlos Romero, Ahmed Sadek, Danielle Belardo, Yasmin Raza and Paul Forfia
J. Clin. Med. 2023, 12(22), 7149; https://doi.org/10.3390/jcm12227149 - 17 Nov 2023
Cited by 1 | Viewed by 1934
Abstract
In patients with right heart failure (RHF) and pulmonary hypertension (PH), classical teaching often advises cautious diuresis in the setting of ‘preload dependence’ to avoid renal injury and hemodynamic compromise. However, while this physiology may hold true in some clinical settings, such as [...] Read more.
In patients with right heart failure (RHF) and pulmonary hypertension (PH), classical teaching often advises cautious diuresis in the setting of ‘preload dependence’ to avoid renal injury and hemodynamic compromise. However, while this physiology may hold true in some clinical settings, such as acute ischemia with right ventricular infarction, it cannot necessarily be extended to PH-related RHF. Rather, in patients with precapillary PH and decompensated RHF, diuresis aimed to decongest the right heart and systemic venous system may be directly beneficial. This study aimed to evaluate the effects of diuresis on renal function and blood pressure in patients with severe precapillary PH. A retrospective chart review was conducted on 62 patients with severe precapillary PH admitted for decompensated RHF. The hemodynamic phenotype of these patients was characterized by invasive hemodynamics and echocardiographic data. Laboratory and hemodynamic data were collected at both admission and discharge. After large-volume diuresis in this patient population, there was an improvement in both glomerular filtration rate and creatinine. While there was a decline in blood pressure after diuresis, this was not clinically significant, given the blood pressure remained in a normal range with improvement in renal function. In conclusion, this study demonstrated that despite concern for preload dependence, significant diuresis in patients with acute decompensated RHF from precapillary PH is not only safe but beneficial. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Updates in Diagnosis and Management)
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10 pages, 521 KiB  
Article
Significance of Hypocapnia in the Risk Assessment of Patients with Pulmonary Hypertension
by Maria Aetou, Lora Wahab, Michael Dreher and Ayham Daher
J. Clin. Med. 2023, 12(19), 6307; https://doi.org/10.3390/jcm12196307 - 30 Sep 2023
Cited by 2 | Viewed by 811
Abstract
Blood gas analysis is part of the diagnostic work−up for pulmonary hypertension (PH). Although some studies have found that the partial pressure of carbon dioxide (PaCO2) is an independent marker of mortality in individuals with pulmonary arterial hypertension (PH Group 1), [...] Read more.
Blood gas analysis is part of the diagnostic work−up for pulmonary hypertension (PH). Although some studies have found that the partial pressure of carbon dioxide (PaCO2) is an independent marker of mortality in individuals with pulmonary arterial hypertension (PH Group 1), there is a lack of data regarding the significance of PaCO2 in individuals with different types of PH based on the new 2022 definitions. Therefore, this study analyzed data from 157 individuals who were undergoing PH work−up, including right heart catheterization, using PH definitions from the 2022 European Society of Cardiology/European Respiratory Society guidelines. At diagnosis, N−terminal pro−B−type natriuretic peptide (NT−pro−BNP) levels were significantly higher, but the time−course of NT−pro−BNP levels during treatment was significantly more favorable in individuals with pulmonary arterial hypertension (PH Group 1) who did versus did not have hypocapnia (p = 0.026 and p = 0.017, respectively). These differences based on the presence of hypocapnia were not seen in individuals with PH Groups 2, 3, or 4. In conclusion, using the new definition of PH, hypocapnia may correlate with worse risk stratification at diagnosis in individuals with pulmonary arterial hypertension. However, hypocapnic individuals with pulmonary arterial hypertension may benefit more from disease−specific therapy than those without hypocapnia. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Updates in Diagnosis and Management)
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12 pages, 789 KiB  
Article
Determinants of Severe Nocturnal Hypoxemia in Adults with Chronic Thromboembolic Pulmonary Hypertension and Sleep-Related Breathing Disorders
by Caner Çınar, Şehnaz Olgun Yıldızeli, Baran Balcan, Bedrettin Yıldızeli, Bülent Mutlu and Yüksel Peker
J. Clin. Med. 2023, 12(14), 4639; https://doi.org/10.3390/jcm12144639 - 12 Jul 2023
Cited by 1 | Viewed by 1023
Abstract
Objectives: We aimed to investigate the occurrence of sleep-related breathing disorders (SRBDs) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and addressed the effect of pulmonary hemodynamics and SRBD indices on the severity of nocturnal hypoxemia (NH). Methods: An overnight polysomnography (PSG) was [...] Read more.
Objectives: We aimed to investigate the occurrence of sleep-related breathing disorders (SRBDs) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and addressed the effect of pulmonary hemodynamics and SRBD indices on the severity of nocturnal hypoxemia (NH). Methods: An overnight polysomnography (PSG) was conducted in patients with CTEPH, who were eligible for pulmonary endarterectomy. Pulmonary hemodynamics (mean pulmonary arterial pressure (mPAP), pulmonary arterial wedge pressure (PAWP), pulmonary vascular resistance (PVR) measured with right heart catheterization (RHC)), PSG variables (apnea–hypopnea index (AHI)), lung function and carbon monoxide diffusion capacity (DLCO) values, as well as demographics and comorbidities were entered into a logistic regression model to address the determinants of severe NH (nocturnal oxyhemoglobin saturation (SpO2) < 90% under >20% of total sleep time (TST)). SRBDs were defined as obstructive sleep apnea (OSA; as an AHI ≥ 15 events/h), central sleep apnea with Cheyne–Stokes respiration (CSA–CSR; CSR pattern ≥ 50% of TST), obesity hypoventilation syndrome (OHS), and isolated sleep-related hypoxemia (ISRH; SpO2 < 88% under >5 min without OSA, CSA, or OHS). Results: In all, 50 consecutive patients (34 men and 16 women; mean age 54.0 (SD 15.1) years) were included. The average mPAP was 43.8 (SD 16.8) mmHg. SRBD was observed in 40 (80%) patients, of whom 27 had OSA, 2 CSA–CSR, and 11 ISRH. None had OHS. Severe NH was observed in 31 (62%) patients. Among the variables tested, age (odds ratio (OR) 1.08, 95% confidence interval [CI] 1.01–1.15; p = 0.031), mPAP (OR 1.11 [95% CI 1.02–1.12; p = 0.012]), and AHI (OR 1.17 [95% CI 1.02–1.35; p = 0.031]) were independent determinants of severe NH. Conclusions: Severe NH is highly prevalent in patients with CTEPH. Early screening for SRBDs and intervention with nocturnal supplemental oxygen and/or positive airway pressure as well as pulmonary endarterectomy may reduce adverse outcomes in patients with CTEPH. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Updates in Diagnosis and Management)
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Review

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15 pages, 3741 KiB  
Review
Arrhythmias in Patients with Pulmonary Hypertension and Right Ventricular Failure: Importance of Rhythm Control Strategies
by Suneesh Anand and Edmond M. Cronin
J. Clin. Med. 2024, 13(7), 1866; https://doi.org/10.3390/jcm13071866 - 24 Mar 2024
Viewed by 3063
Abstract
Arrhythmias frequently complicate the course of advanced pulmonary hypertension, often leading to hemodynamic compromise, functional impairment, and mortality. Given the importance of right atrial function in this physiology, the restoration and maintenance of sinus rhythm are of critical importance. In this review, we [...] Read more.
Arrhythmias frequently complicate the course of advanced pulmonary hypertension, often leading to hemodynamic compromise, functional impairment, and mortality. Given the importance of right atrial function in this physiology, the restoration and maintenance of sinus rhythm are of critical importance. In this review, we outline the pathophysiology of arrhythmias and their impact on right heart performance; describe considerations for antiarrhythmic drug selection, anesthetic and periprocedural management; and discuss the results of catheter ablation techniques in this complex and challenging patient population. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Updates in Diagnosis and Management)
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9 pages, 729 KiB  
Review
Exercise Testing in Patients with Pulmonary Hypertension
by Anika Vaidy, Cyrus A. Vahdatpour and Jeremy Mazurek
J. Clin. Med. 2024, 13(3), 795; https://doi.org/10.3390/jcm13030795 - 30 Jan 2024
Viewed by 816
Abstract
Pulmonary hypertension (PH), defined by a mean pulmonary artery pressure of >20 mm Hg, often presents with non-specific symptoms such as dyspnea and exercise intolerance, making it difficult to diagnose early before the onset of right heart dysfunction. Therefore, exercise testing can be [...] Read more.
Pulmonary hypertension (PH), defined by a mean pulmonary artery pressure of >20 mm Hg, often presents with non-specific symptoms such as dyspnea and exercise intolerance, making it difficult to diagnose early before the onset of right heart dysfunction. Therefore, exercise testing can be of great utility for clinicians who are evaluating patients with an unclear etiology of exercise intolerance by helping identify the underlying mechanisms of their disease. The presence of PH is associated with adverse clinical outcomes, with distinct differences and patterns in the cardiovascular and ventilatory responses to exercise across various PH phenotypes. We discuss the role of exercise-invasive hemodynamic testing, cardiopulmonary exercise testing, and exercise stress echocardiography modalities across the spectrum of PH. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Updates in Diagnosis and Management)
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13 pages, 2215 KiB  
Review
Post-Capillary Pulmonary Hypertension: Clinical Review
by Joshua M. Riley, James J. Fradin, Douglas H. Russ, Eric D. Warner, Yevgeniy Brailovsky and Indranee Rajapreyar
J. Clin. Med. 2024, 13(2), 625; https://doi.org/10.3390/jcm13020625 - 22 Jan 2024
Viewed by 2956
Abstract
Pulmonary hypertension (PH) caused by left heart disease, also known as post-capillary PH, is the most common etiology of PH. Left heart disease due to systolic dysfunction or heart failure with preserved ejection fraction, valvular heart disease, and left atrial myopathy due to [...] Read more.
Pulmonary hypertension (PH) caused by left heart disease, also known as post-capillary PH, is the most common etiology of PH. Left heart disease due to systolic dysfunction or heart failure with preserved ejection fraction, valvular heart disease, and left atrial myopathy due to atrial fibrillation are causes of post-capillary PH. Elevated left-sided filling pressures cause pulmonary venous congestion due to backward transmission of pressures and post-capillary PH. In advanced left-sided heart disease or valvular heart disease, chronic uncontrolled venous congestion may lead to remodeling of the pulmonary arterial system, causing combined pre-capillary and post-capillary PH. The hemodynamic definition of post-capillary PH includes a mean pulmonary arterial pressure > 20 mmHg, pulmonary vascular resistance < 3 Wood units, and pulmonary capillary wedge pressure > 15 mmHg. Echocardiography is important in the identification and management of the underlying cause of post-capillary PH. Management of post-capillary PH is focused on the treatment of the underlying condition. Strategies are geared towards pharmacotherapy and guideline-directed medical therapy for heart failure, surgical or percutaneous management of valvular disorders, and control of modifiable risk factors and comorbid conditions. Referral to centers with advanced heart and pulmonary teams has shown to improve morbidity and mortality. There is emerging interest in the use of targeted agents classically used in pulmonary arterial hypertension, but current data remain limited and conflicting. This review aims to serve as a comprehensive summary of postcapillary PH and its etiologies, pathophysiology, diagnosis, and management, particularly as it pertains to advanced heart failure. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Updates in Diagnosis and Management)
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13 pages, 656 KiB  
Review
Pulmonary Hypertension in Underrepresented Minorities: A Narrative Review
by Johanna Contreras, Jeremy Nussbaum, Peter Cangialosi, Sahityasri Thapi, Ankitha Radakrishnan, Jillian Hall, Prashasthi Ramesh, Maria Giovanna Trivieri and Alejandro Folch Sandoval
J. Clin. Med. 2024, 13(1), 285; https://doi.org/10.3390/jcm13010285 - 4 Jan 2024
Viewed by 1137
Abstract
Minoritized racial and ethnic groups suffer disproportionately from the incidence and morbidity of pulmonary hypertension (PH), as well as its associated cardiovascular, pulmonary, and systemic conditions. These disparities are largely explained by social determinants of health, including access to care, systemic biases, socioeconomic [...] Read more.
Minoritized racial and ethnic groups suffer disproportionately from the incidence and morbidity of pulmonary hypertension (PH), as well as its associated cardiovascular, pulmonary, and systemic conditions. These disparities are largely explained by social determinants of health, including access to care, systemic biases, socioeconomic status, and environment. Despite this undue burden, minority patients remain underrepresented in PH research. Steps should be taken to mitigate these disparities, including initiatives to increase research participation, combat inequities in access to care, and improve the treatment of the conditions associated with PH. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Updates in Diagnosis and Management)
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14 pages, 10828 KiB  
Review
Diagnostic Evaluation of Pulmonary Hypertension: A Comprehensive Approach for Primary Care Physicians
by Suneesh Anand, Ahmed Sadek, Anjali Vaidya and Estefania Oliveros
J. Clin. Med. 2023, 12(23), 7309; https://doi.org/10.3390/jcm12237309 - 25 Nov 2023
Viewed by 3271
Abstract
Pulmonary hypertension (PH) is a disorder involving a heterogeneous group of medical conditions encompassing several cardiopulmonary illnesses. Implementing new diagnostic criteria for PH in conjunction with multimodality diagnostic tools is crucial for accurate and early recognition of this life-threatening form of right heart [...] Read more.
Pulmonary hypertension (PH) is a disorder involving a heterogeneous group of medical conditions encompassing several cardiopulmonary illnesses. Implementing new diagnostic criteria for PH in conjunction with multimodality diagnostic tools is crucial for accurate and early recognition of this life-threatening form of right heart failure. This should streamline early referrals to accredited PH centers, with a goal to rapidly institute targeted therapy in order to optimize prognosis. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Updates in Diagnosis and Management)
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