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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 -
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 -
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 -
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 Lancet. Respiratory Medicine Sep 2023Patients with chronic lung diseases, particularly interstitial lung disease and chronic obstructive pulmonary disease, frequently develop pulmonary hypertension, which... (Review)
Review
Patients with chronic lung diseases, particularly interstitial lung disease and chronic obstructive pulmonary disease, frequently develop pulmonary hypertension, which results in clinical deterioration, worsening of oxygen uptake, and an increased mortality risk. Pulmonary hypertension can develop and progress independently from the underlying lung disease. The pulmonary vasculopathy is distinct from that of other forms of pulmonary hypertension, with vascular ablation due to loss of small pulmonary vessels being a key feature. Long-term tobacco exposure might contribute to this type of pulmonary vascular remodelling. The distinct pathomechanisms together with the underlying lung disease might explain why treatment options for this condition remain scarce. Most drugs approved for pulmonary arterial hypertension have shown no or sometimes harmful effects in pulmonary hypertension associated with lung disease. An exception is inhaled treprostinil, which improves exercise capacity in patients with interstitial lung disease and pulmonary hypertension. There is a pressing need for safe, effective treatment options and for reliable, non-invasive diagnostic tools to detect and characterise pulmonary hypertension in patients with chronic lung disease.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Disease, Chronic Obstructive; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Clinical Deterioration
PubMed: 37591300
DOI: 10.1016/S2213-2600(23)00259-X -
Seminars in Fetal & Neonatal Medicine Aug 2022A disruption in the well-orchestrated fetal-to-neonatal cardiopulmonary transition at birth results in the clinical conundrum of severe hypoxemic respiratory failure... (Review)
Review
A disruption in the well-orchestrated fetal-to-neonatal cardiopulmonary transition at birth results in the clinical conundrum of severe hypoxemic respiratory failure associated with elevated pulmonary vascular resistance (PVR), referred to as persistent pulmonary hypertension of the newborn (PPHN). In the past three decades, the advent of surfactant, newer modalities of ventilation, inhaled nitric oxide, other pulmonary vasodilators, and finally extracorporeal membrane oxygenation (ECMO) have made giant strides in improving the outcomes of infants with PPHN. However, death or the need for ECMO occurs in 10-20% of term infants with PPHN. Better understanding of the etiopathogenesis of PPHN can lead to physiology-driven management strategies. This manuscript reviews the fetal circulation, cardiopulmonary transition at birth, etiology, and pathophysiology of PPHN.
Topics: Extracorporeal Membrane Oxygenation; Humans; Hypertension, Pulmonary; Infant; Infant, Newborn; Nitric Oxide; Persistent Fetal Circulation Syndrome; Vascular Resistance
PubMed: 35963740
DOI: 10.1016/j.siny.2022.101381 -
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 -
The American Journal of Managed Care Mar 2021Group 1 pulmonary hypertension (or pulmonary arterial hypertension) is a rare, highly complex, and progressive disorder that is incurable and ultimately can lead to... (Review)
Review
Group 1 pulmonary hypertension (or pulmonary arterial hypertension) is a rare, highly complex, and progressive disorder that is incurable and ultimately can lead to premature death. PAH causes significant physical, social, work, and emotional burdens among affected patients and their caregivers. Early diagnosis and initiation of treatment is required for best outcomes; however, the clinical presentation of PAH is nonspecific and frequently overlaps with several other conditions, often leading to a delay in diagnosis or misdiagnosis. In the past decades, increased understanding of the pathobiology of PAH has led to changes in its definition. Additionally, contemporary PAH registries have shown greater survival rates among patients with PAH and have allowed for the development of risk calculator tools that are now used to drive therapeutic goals. To date, multiple PAH-specific therapies have been developed, and all currently target one of 3 pathways that contribute to the endothelial dysfunction pathogenesis of PAH (prostacyclin, endothelin, and nitric oxide pathways). Because PAH is classified into 7 subgroups, it is essential that individuals are grouped appropriately for the efficacy of treatment and avoidance of harm. As health-related quality of life for PAH is multifactorial, it is important that patients are involved in the clinical decision-making process and have access to multidisciplinary care. The purpose of this review is to update healthcare professionals on the management of PAH with the most current information on epidemiology, pathophysiology, clinical presentation, and diagnostic considerations.
Topics: Epoprostenol; Humans; Hypertension, Pulmonary; Prostaglandins I; Pulmonary Arterial Hypertension; Quality of Life
PubMed: 33710842
DOI: 10.37765/ajmc.2021.88609