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Presse Medicale (Paris, France : 1983) Sep 2023Pulmonary arterial hypertension (PAH) is a rare and progressive disease characterised by remodelling of the pulmonary arteries and progressive narrowing of the pulmonary... (Review)
Review
Pulmonary arterial hypertension (PAH) is a rare and progressive disease characterised by remodelling of the pulmonary arteries and progressive narrowing of the pulmonary vasculature. This leads to a progressive increase in pulmonary vascular resistance and pulmonary arterial pressure and, if left untreated, to right ventricular failure and death. A correct diagnosis requires a complete work-up including right heart catheterisation performed in a specialised centre. Although our knowledge of the epidemiology, pathology and pathophysiology of the disease, as well as the development of innovative therapies, has progressed in recent decades, PAH remains a serious clinical condition. Current treatments for the disease target the three specific pathways of endothelial dysfunction that characterise PAH: the endothelin, nitric oxide and prostacyclin pathways. The current treatment algorithm is based on the assessment of severity using a multiparametric risk stratification approach at the time of diagnosis (baseline) and at regular follow-up visits. It recommends the initiation of combination therapy in PAH patients without cardiopulmonary comorbidities. The choice of therapy (dual or triple) depends on the initial severity of the condition. The main treatment goal is to achieve low-risk status. Further escalation of treatment is required if low-risk status is not achieved at subsequent follow-up assessments. In the most severe patients, who are already on maximal medical therapy, lung transplantation may be indicated. Recent advances in understanding the pathophysiology of the disease have led to the development of promising emerging therapies targeting dysfunctional pathways beyond endothelial dysfunction, including the TGF-β and PDGF pathways.
Topics: Humans; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Lung Transplantation; Antihypertensive Agents
PubMed: 37516248
DOI: 10.1016/j.lpm.2023.104168 -
Jornal Brasileiro de Pneumologia :... Oct 2023
Topics: Humans; Hypertension, Pulmonary; Hypertension; Lung
PubMed: 37909553
DOI: 10.36416/1806-3756/e20230275 -
Clinics in Chest Medicine Mar 2024Pulmonary hypertension is a life-threatening complication of advanced sarcoidosis. Many mechanisms can cause an elevation of pulmonary pressure in sarcoidosis, leading... (Review)
Review
Pulmonary hypertension is a life-threatening complication of advanced sarcoidosis. Many mechanisms can cause an elevation of pulmonary pressure in sarcoidosis, leading to precapillary or postcapillary pulmonary hypertension. Sarcoidosis-associated pulmonary hypertension contributes to severe exertional dyspnea, reduced exercise capacity, and notably compromised the survival. Despite the critical functional and prognostic implications of pulmonary hypertension in sarcoidosis, there is a scarcity of specific guidelines on the management of these patients due to a lack of evidence. Hence, further research is required to identify subgroups of patients who may benefit from pulmonary arterial hypertension-targeted therapies and/or immunosuppressive therapies.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Sarcoidosis; Lung; Prognosis; Sarcoidosis, Pulmonary
PubMed: 38245366
DOI: 10.1016/j.ccm.2023.08.007 -
European Heart Journal Aug 2023The current treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) as depicted in the 2022 European Society of Cardiology (ESC)/European...
Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: a clinical consensus statement of the ESC working group on pulmonary circulation and right ventricular function.
The current treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) as depicted in the 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines on the diagnosis and treatment of pulmonary hypertension (PH) includes a multimodal approach of combinations of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapies to target major vessel pulmonary vascular lesions, and microvasculopathy. Today, BPA of >1700 patients has been reported in the literature from centers in Asia, the US, and also Europe; many more patients have been treated outside literature reports. As BPA becomes part of routine care of patients with CTEPH, benchmarks for safe and effective care delivery become increasingly important. In light of this development, the ESC Working Group on Pulmonary Circulation and Right Ventricular Function has decided to publish a document that helps standardize BPA to meet the need of uniformity in patient selection, procedural planning, technical approach, materials and devices, treatment goals, complications including their management, and patient follow-up, thus complementing the guidelines. Delphi methodology was utilized for statements that were not evidence based. First, an anatomical nomenclature and a description of vascular lesions are provided. Second, treatment goals and definitions of complete BPA are outlined. Third, definitions of complications are presented which may be the basis for a standardized reporting in studies involving BPA. The document is intended to serve as a companion to the official ESC/ERS guidelines.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Embolism; Pulmonary Circulation; Ventricular Function, Right; Angioplasty, Balloon; Pulmonary Artery; Chronic Disease; Cardiology
PubMed: 37470202
DOI: 10.1093/eurheartj/ehad413 -
Nature Reviews. Disease Primers Jan 2024
Topics: Humans; Hypertension, Pulmonary; Hypertension; Lung
PubMed: 38177187
DOI: 10.1038/s41572-023-00491-w -
American Journal of Perinatology Oct 2023Persistent pulmonary hypertension of the newborn, or PPHN, represents a challenging condition associated with high morbidity and mortality. Management is complicated by...
Persistent pulmonary hypertension of the newborn, or PPHN, represents a challenging condition associated with high morbidity and mortality. Management is complicated by complex pathophysiology and limited neonatal specific evidence-based literature, leading to a lack of universal contemporary clinical guidelines for the care of these patients. To address this need and to provide consistent high-quality clinical care for this challenging population in our neonatal intensive care unit, we sought to develop a comprehensive clinical guideline for the acute stabilization and management of neonates with PPHN. Utilizing cross-disciplinary expertise and incorporating an extensive literature search to guide best practice, we present an approachable, pragmatic, and clinically relevant guide for the bedside management of acute PPHN. KEY POINTS: · PPHN is associated with several unique diagnoses; the associated pathophysiology is different for each unique diagnosis.. · PPHN is a challenging, dynamic, and labile process for which optimal care requires frequent reassessment.. · Key management goals are adequate tissue oxygen delivery, avoiding harm..
Topics: Infant, Newborn; Humans; Persistent Fetal Circulation Syndrome; Hypertension, Pulmonary; Intensive Care Units, Neonatal
PubMed: 34852367
DOI: 10.1055/a-1711-0778 -
Anatolian Journal of Cardiology Nov 2023
Topics: Humans; Hypertension, Pulmonary; Hypertension; Algorithms; Machine Learning
PubMed: 37909349
DOI: 10.14744/AnatolJCardiol.2023.11 -
The European Respiratory Journal Sep 2023
Topics: Humans; Heart Ventricles; Pulmonary Arterial Hypertension; Heart; Familial Primary Pulmonary Hypertension; Hemodynamics
PubMed: 37696566
DOI: 10.1183/13993003.01513-2023 -
International Journal of Molecular... Jan 2024Pulmonary hypertension (PH) is recognized as a pathophysiological disorder encompassing a wide spectrum of clinical conditions related to various cardiovascular and...
Pulmonary hypertension (PH) is recognized as a pathophysiological disorder encompassing a wide spectrum of clinical conditions related to various cardiovascular and respiratory diseases [...].
Topics: Humans; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Hypertension, Pulmonary
PubMed: 38256236
DOI: 10.3390/ijms25021166 -
Clinics in Perinatology Mar 2024
Topics: Infant, Newborn; Humans; Hypertension, Pulmonary; Hypertension; Phenotype
PubMed: 38325950
DOI: 10.1016/j.clp.2023.11.010