-
European Heart Journal Oct 2022
Topics: Endothelin Receptor Antagonists; Humans; Hypertension, Pulmonary
PubMed: 36017548
DOI: 10.1093/eurheartj/ehac237 -
American Journal of Respiratory and... Sep 2023Major advances in pulmonary arterial hypertension, pulmonary hypertension (PH) associated with lung disease, and chronic thromboembolic PH cast new light on the... (Review)
Review
Major advances in pulmonary arterial hypertension, pulmonary hypertension (PH) associated with lung disease, and chronic thromboembolic PH cast new light on the pathogenetic mechanisms, epidemiology, diagnostic approach, and therapeutic armamentarium for pulmonary vascular disease. Here, we summarize key basic, translational, and clinical PH reports, emphasizing findings that build on current state-of-the-art research. This review includes cutting-edge progress in translational pulmonary vascular biology, with a guide to the diagnosis of patients in clinical practice, incorporating recent PH definition revisions that continue emphasis on early detection of disease. PH management is reviewed including an overview of the evolving considerations for the approach to treatment of PH in patients with cardiopulmonary comorbidities, as well as a discussion of the groundbreaking sotatercept data for the treatment of pulmonary arterial hypertension.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Vascular Diseases; Lung; Familial Primary Pulmonary Hypertension; Chronic Disease; Pulmonary Embolism
PubMed: 37450768
DOI: 10.1164/rccm.202302-0327SO -
Annals of Internal Medicine Apr 2021Pulmonary hypertension is the term used to describe a group of disorders characterized by abnormally high pressures in the pulmonary arteries. Initial evaluation is... (Review)
Review
Pulmonary hypertension is the term used to describe a group of disorders characterized by abnormally high pressures in the pulmonary arteries. Initial evaluation is focused on identifying the cause, which helps guide appropriate treatment. Pulmonary hypertension is often a feature of advanced common diseases, such as chronic obstructive pulmonary disease and left heart disease, and treatment is focused primarily on the underlying disease. More rarely, pulmonary hypertension results from chronic organized thromboemboli or a primary vasculopathy. The former requires evaluation for surgical intervention, and the latter is treated with advanced medical therapies.
Topics: Diagnosis, Differential; Humans; Hypertension, Pulmonary; Risk Factors
PubMed: 33844574
DOI: 10.7326/AITC202104200 -
BMJ (Clinical Research Ed.) Mar 2018Pulmonary hypertension is defined as a resting mean pulmonary artery pressure of 25 mm Hg or above. This review deals with pulmonary arterial hypertension (PAH), a type... (Review)
Review
Pulmonary hypertension is defined as a resting mean pulmonary artery pressure of 25 mm Hg or above. This review deals with pulmonary arterial hypertension (PAH), a type of pulmonary hypertension that primarily affects the pulmonary vasculature. In PAH, the pulmonary vasculature is dynamically obstructed by vasoconstriction, structurally obstructed by adverse vascular remodeling, and pathologically non-compliant as a result of vascular fibrosis and stiffening. Many cell types are abnormal in PAH, including vascular cells (endothelial cells, smooth muscle cells, and fibroblasts) and inflammatory cells. Progress has been made in identifying the causes of PAH and approving new drug therapies. A cancer-like increase in cell proliferation and resistance to apoptosis reflects acquired abnormalities of mitochondrial metabolism and dynamics. Mutations in the type II bone morphogenetic protein receptor ( gene dramatically increase the risk of developing heritable PAH. Epigenetic dysregulation of DNA methylation, histone acetylation, and microRNAs also contributes to disease pathogenesis. Aberrant bone morphogenetic protein signaling and epigenetic dysregulation in PAH promote cell proliferation in part through induction of a Warburg mitochondrial-metabolic state of uncoupled glycolysis. Complex changes in cytokines (interleukins and tumor necrosis factor), cellular immunity (T lymphocytes, natural killer cells, macrophages), and autoantibodies suggest that PAH is, in part, an autoimmune, inflammatory disease. Obstructive pulmonary vascular remodeling in PAH increases right ventricular afterload causing right ventricular hypertrophy. In some patients, maladaptive changes in the right ventricle, including ischemia and fibrosis, reduce right ventricular function and cause right ventricular failure. Patients with PAH have dyspnea, reduced exercise capacity, exertional syncope, and premature death from right ventricular failure. PAH targeted therapies (prostaglandins, phosphodiesterase-5 inhibitors, endothelin receptor antagonists, and soluble guanylate cyclase stimulators), used alone or in combination, improve functional capacity and hemodynamics and reduce hospital admissions. However, these vasodilators do not target key features of PAH pathogenesis and have not been shown to reduce mortality, which remains about 50% at five years. This review summarizes the epidemiology, pathogenesis, diagnosis, and treatment of PAH.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Artery; Ventricular Function, Right
PubMed: 29540357
DOI: 10.1136/bmj.j5492 -
Medicina Clinica Jun 2022Pulmonary arterial hypertension (PAH) is a rare disease characterized by adverse remodeling of the arterial tree leading to increased vascular resistance with subsequent... (Review)
Review
Pulmonary arterial hypertension (PAH) is a rare disease characterized by adverse remodeling of the arterial tree leading to increased vascular resistance with subsequent increase in right ventricular afterload and eventual development of heart failure. The nonspecific clinical manifestations and lack of knowledge of pathology lead to a poor prognosis associated with delay in diagnosis and initiation of treatment. The most recent recommendations focus on optimizing the early differential diagnosis with other causes of pulmonary hypertension to initiate appropriate treatment based on the mortality risk estimation. In the last years, with the improvement in the diagnostic process, the emergence of new specific treatments, and the creation of specialized referral units for this pathology, the prognosis, and quality of life of patients with PAH have improved significantly.
Topics: Heart Failure; Heart Ventricles; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Quality of Life
PubMed: 35279313
DOI: 10.1016/j.medcli.2022.01.003 -
The European Respiratory Journal Jan 2019Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure... (Review)
Review
Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest, measured by right heart catheterisation. Recent data from normal subjects has shown that normal mPAP was 14.0±3.3 mmHg. Two standard deviations above this mean value would suggest mPAP >20 mmHg as above the upper limit of normal (above the 97.5th percentile). This definition is no longer arbitrary, but based on a scientific approach. However, this abnormal elevation of mPAP is not sufficient to define pulmonary vascular disease as it can be due to an increase in cardiac output or pulmonary arterial wedge pressure. Thus, this 6th WSPH Task Force proposes to include pulmonary vascular resistance ≥3 Wood Units in the definition of all forms of pre-capillary PH associated with mPAP >20 mmHg. Prospective trials are required to determine whether this PH population might benefit from specific management.Regarding clinical classification, the main Task Force changes were the inclusion in group 1 of a subgroup "pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers", due to the specific prognostic and management of these patients, and a subgroup "PAH with overt features of venous/capillaries (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement", due to evidence suggesting a continuum between arterial, capillary and vein involvement in PAH.
Topics: Consensus Development Conferences as Topic; Heart Diseases; Hemodynamics; Humans; Hypertension, Pulmonary
PubMed: 30545968
DOI: 10.1183/13993003.01913-2018 -
Journal of Veterinary Internal Medicine Mar 2020Pulmonary hypertension (PH), defined by increased pressure within the pulmonary vasculature, is a hemodynamic and pathophysiologic state present in a wide variety of...
Pulmonary hypertension (PH), defined by increased pressure within the pulmonary vasculature, is a hemodynamic and pathophysiologic state present in a wide variety of cardiovascular, respiratory, and systemic diseases. The purpose of this consensus statement is to provide a multidisciplinary approach to guidelines for the diagnosis, classification, treatment, and monitoring of PH in dogs. Comprehensive evaluation including consideration of signalment, clinical signs, echocardiographic parameters, and results of other diagnostic tests supports the diagnosis of PH and allows identification of associated underlying conditions. Dogs with PH can be classified into the following 6 groups: group 1, pulmonary arterial hypertension; group 2, left heart disease; group 3, respiratory disease/hypoxia; group 4, pulmonary emboli/pulmonary thrombi/pulmonary thromboemboli; group 5, parasitic disease (Dirofilaria and Angiostrongylus); and group 6, disorders that are multifactorial or with unclear mechanisms. The approach to treatment of PH focuses on strategies to decrease the risk of progression, complications, or both, recommendations to target underlying diseases or factors contributing to PH, and PH-specific treatments. Dogs with PH should be monitored for improvement, static condition, or progression, and any identified underlying disorder should be addressed and monitored simultaneously.
Topics: Animals; Dog Diseases; Dogs; Hypertension, Pulmonary; Practice Guidelines as Topic; Societies, Scientific; Veterinary Medicine
PubMed: 32065428
DOI: 10.1111/jvim.15725 -
Mayo Clinic Proceedings Sep 2020Pulmonary hypertension (PH) is classified into 5 clinical subgroups: pulmonary arterial hypertension (PAH), PH due to left-sided heart disease, PH due to chronic lung... (Review)
Review
Pulmonary hypertension (PH) is classified into 5 clinical subgroups: pulmonary arterial hypertension (PAH), PH due to left-sided heart disease, PH due to chronic lung disease, chronic thromboembolic PH (CTEPH), and PH with an unclear and/or multifactorial mechanisms. A range of underlying conditions can lead to these disorders. Overall, PH affects approximately 1% of the global population, and over half of patients with heart failure may be affected. Cardiologists are therefore likely to encounter PH in their practice. Routine tests in patients with symptoms and physical findings suggestive of PH include electrocardiography, chest radiography, and pulmonary function tests. Transthoracic echocardiography is used to estimate the probability of PH. All patients with suspected or confirmed PH, without confirmed left-sided heart or lung diseases, should have a ventilation-perfusion scan to exclude CTEPH. Right-sided heart catheterization is essential for accurate diagnosis and classification. All patients with PAH or CTEPH must be referred to a specialist center. Surgical pulmonary endarterectomy is the treatment of choice for eligible patients with CTEPH. Targeted treatments (phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogues, and prostacyclin receptor agonists) are licensed for patients with PAH. The soluble guanylate cyclase stimulator riociguat is the only licensed targeted therapy for patients with inoperable or persistent/recurrent CTEPH. Management of PH resulting from left-sided heart disease primarily involves treatment of the underlying condition.
Topics: Cardiac Catheterization; Electrocardiography; Female; Humans; Hypertension, Pulmonary; Male; Risk Factors
PubMed: 32861339
DOI: 10.1016/j.mayocp.2020.04.039 -
The Medical Clinics of North America May 2019Pulmonary hypertension (PH) is a chronic and progressive disease that presents like many other lung diseases, often leading to a delay in diagnosis, and therefore a... (Review)
Review
Pulmonary hypertension (PH) is a chronic and progressive disease that presents like many other lung diseases, often leading to a delay in diagnosis, and therefore a delay in optimal therapy. This article provides a review of PH for internists, covering clinical presentation, diagnostic algorithm, different types of PH, and overview of treatments. In addition, it emphasizes the importance of early referral to, and partnership between, PH specialists and physicians on the front lines to improve early diagnosis and optimize management of these complex patients.
Topics: Humans; Hypertension, Pulmonary; Referral and Consultation; Risk Assessment; Ventricular Dysfunction, Right
PubMed: 30955510
DOI: 10.1016/j.mcna.2018.12.002 -
Nature Reviews. Disease Primers Jan 2024Pulmonary hypertension encompasses a range of conditions directly or indirectly leading to elevated pressures within the pulmonary arteries. Five main groups of... (Review)
Review
Pulmonary hypertension encompasses a range of conditions directly or indirectly leading to elevated pressures within the pulmonary arteries. Five main groups of pulmonary hypertension are recognized, all defined by a mean pulmonary artery pressure of >20 mmHg: pulmonary arterial hypertension (rare), pulmonary hypertension associated with left-sided heart disease (very common), pulmonary hypertension associated with lung disease (common), pulmonary hypertension associated with pulmonary artery obstructions, usually related to thromboembolic disease (rare), and pulmonary hypertension with unclear and/or multifactorial mechanisms (rare). At least 1% of the world's population is affected, with a greater burden more likely in low-income and middle-income countries. Across all its forms, pulmonary hypertension is associated with adverse vascular remodelling with obstruction, stiffening and vasoconstriction of the pulmonary vasculature. Without proactive management this leads to hypertrophy and ultimately failure of the right ventricle, the main cause of death. In older individuals, dyspnoea is the most common symptom. Stepwise investigation precedes definitive diagnosis with right heart catheterization. Medical and surgical treatments are approved for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. There are emerging treatments for other forms of pulmonary hypertension; but current therapy primarily targets the underlying cause. There are still major gaps in basic, clinical and translational knowledge; thus, further research, with a focus on vulnerable populations, is needed to better characterize, detect and effectively treat all forms of pulmonary hypertension.
Topics: Humans; Aged; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Pulmonary Artery; Lung
PubMed: 38177157
DOI: 10.1038/s41572-023-00486-7