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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 -
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 -
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 -
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 -
European Respiratory Review : An... Mar 2016In 2015, more than 800 papers were published in the field of pulmonary hypertension. A Clinical Year in Review article cannot possibly incorporate all this work and... (Review)
Review
In 2015, more than 800 papers were published in the field of pulmonary hypertension. A Clinical Year in Review article cannot possibly incorporate all this work and needs to be selective. The recently published European guidelines for the diagnosis and treatment of pulmonary hypertension contain an inclusive summary of all published clinical studies conducted until very recently. Here, we provide an overview of papers published after the finalisation of the guideline. In addition, we summarise recent advances in pulmonary vasculature science. The selection we made from the enormous amount of published work undoubtedly reflects our personal views and may not include all papers with a significant impact in the near or more distant future. The focus of this paper is on the diagnosis of pulmonary arterial hypertension, understanding the success of combination therapy on the right ventricle and scientific breakthroughs.
Topics: Animals; Humans; Hypertension, Pulmonary; Risk Factors; Treatment Outcome
PubMed: 26929415
DOI: 10.1183/16000617.0096-2015 -
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 -
Deutsches Arzteblatt International Feb 2017About 1% of adults suffer from pulmonary hypertension (PH). The various types of PH differ widely with respect to their incidence, clinical significance, and treatment. (Review)
Review
BACKGROUND
About 1% of adults suffer from pulmonary hypertension (PH). The various types of PH differ widely with respect to their incidence, clinical significance, and treatment.
METHODS
Selective review of the literature in association with a consensus conference.
RESULTS
Pulmonary hypertension is divided into five major categories. Those that are of particular clinical relevance are pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension due to left heart and lung diseases. Ten drugs from five different substance classes are now available for the treatment of PH and are often given in combination. The treatment strategy is determined by risk stratification based on the severity of disease, along with the clinical phenotype and possible accompanying illnesses. The preferred treatment for chronic thromboembolic pulmonary hypertension is surgical pulmonary endarterectomy; inoperable patients are treated with drugs and endovascular interventions. PH due to left heart and lung diseases generally calls for specific treatment of pulmonary hypertension only if there is severe right-heart strain.
CONCLUSION
The diagnosis and treatment of severe forms of PH, in particular, pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, are complex and are best carried out in close cooperation between the local physician and specialized centers.
Topics: Adult; Chronic Disease; Endarterectomy; Humans; Hypertension, Pulmonary; Pulmonary Embolism
PubMed: 28241922
DOI: 10.3238/arztebl.2017.0073 -
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 -
Future Cardiology Jul 2018Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg. We focus on its relevance in congenital heart disease, reviewing pathophysiology,... (Review)
Review
Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg. We focus on its relevance in congenital heart disease, reviewing pathophysiology, diagnosis and management. Pulmonary hypertension is a relatively common complication of congenital heart disease, with adult prevalence between 5 and 10%. A multifactorial cause is recognized, relating to the size and nature of cardiac defect as well as environmental and genetic factors. More complex disease is increasingly recognized rather than pure Eisenmenger complex. Remodeling of the pulmonary vascular bed causes increased pulmonary vascular resistance diagnosed by a collection of investigations including echocardiography, exercise testing, cardiac catheterization, MRI and CT scanning. Management employs disease-modifying medications which are now used with increasing benefit.
Topics: Heart Defects, Congenital; Humans; Hypertension, Pulmonary
PubMed: 29792339
DOI: 10.2217/fca-2017-0065