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European Respiratory Review : An... Sep 2021Pulmonary hypertension (PH) commonly affects patients with systemic sclerosis (SSc) and is associated with significant morbidity and increased mortality. PH is a... (Review)
Review
Pulmonary hypertension (PH) commonly affects patients with systemic sclerosis (SSc) and is associated with significant morbidity and increased mortality. PH is a heterogenous condition and several different forms can be associated with SSc, including pulmonary arterial hypertension (PAH) resulting from a pulmonary arterial vasculopathy, PH due to left heart disease and PH due to interstitial lung disease. The incidence of pulmonary veno-occlusive disease is also increased. Accurate and early diagnosis to allow optimal treatment is, therefore, essential. Recent changes to diagnostic haemodynamic criteria at the 6th World Symposium on Pulmonary Hypertension have resulted in therapeutic uncertainty regarding patients with borderline pulmonary haemodynamics. Furthermore, the optimal pulmonary vascular resistance threshold for diagnosing PAH and the role of exercise in identifying early disease require further elucidation. In this article we review the epidemiology, diagnosis, outcomes and treatment of the spectrum of pulmonary vascular phenotypes associated with SSc.
Topics: Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Phenotype; Pulmonary Arterial Hypertension; Scleroderma, Systemic
PubMed: 34407977
DOI: 10.1183/16000617.0053-2021 -
Clinical Medicine (London, England) Sep 2023Pulmonary hypertension (PH) is common, with an estimated prevalence of approximately 1% that increases with age. Prompt and accurate diagnosis is key to institute timely... (Review)
Review
Pulmonary hypertension (PH) is common, with an estimated prevalence of approximately 1% that increases with age. Prompt and accurate diagnosis is key to institute timely and appropriate therapy to improve symptoms and prognosis. The international guidelines for the diagnosis and management of PH have recently been updated, with a lowering of the haemodynamic threshold for diagnosis to a mean pulmonary artery pressure >20 mmHg. New diagnostic algorithms and revised indications for screening in at-risk groups have been developed to facilitate early referral to specialist PH centres. This includes fast-track referral pathways for patients who are either clinically high-risk or are at-risk for pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH). This review summarises key changes in the PH guidelines for general physicians who are, most often, the first healthcare professionals to encounter these patients and consequently have a key role as referrers into specialist PH services.
Topics: Humans; Hypertension, Pulmonary; Prognosis; Physicians; Chronic Disease
PubMed: 37775164
DOI: 10.7861/clinmed.2023-23.5.Cardio4 -
Cardiology Clinics Feb 2022
Topics: Humans; Hypertension; Hypertension, Pulmonary
PubMed: 34809921
DOI: 10.1016/j.ccl.2021.09.002 -
Journal of Cardiothoracic and Vascular... Jun 2022The World Symposium on Pulmonary Hypertension (WSPH) was organized by the World Health Organization in 1973 in response to an increase in pulmonary arterial hypertension...
The World Symposium on Pulmonary Hypertension (WSPH) was organized by the World Health Organization in 1973 in response to an increase in pulmonary arterial hypertension in Europe caused by aminorex, an appetite suppressant. The mandate of this meeting was to review the latest clinical and scientific research and to formulate recommendations to improve the diagnosis and management of pulmonary hypertension (PH). Since 1998, the WSPH has met every five years and in 2018, the sixth annual WSPH revised the hemodynamic definition of PH. This two-part series will review the updated definition, classification, pathophysiology, presentation, diagnosis, management, and perioperative management of patients with PH. In the first part of this series, the definition, classification, pathophysiology, and presentation will be reviewed.
Topics: Europe; Hemodynamics; Humans; Hypertension, Pulmonary
PubMed: 34344595
DOI: 10.1053/j.jvca.2021.06.036 -
Herz Aug 2023
Topics: Humans; Hypertension, Pulmonary; Hypertension; Lung
PubMed: 37525084
DOI: 10.1007/s00059-023-05188-0 -
The Lancet. Respiratory Medicine Sep 2023Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. Important advances have enabled better understanding,... (Review)
Review
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. Important advances have enabled better understanding, characterisation, and treatment of this condition. Guidelines recommending systematic follow-up after acute pulmonary embolism, and the insight that CTEPH can mimic acute pulmonary embolism on initial presentation, have led to the definition of CTEPH imaging characteristics, the introduction of artificial intelligence diagnosis pathways, and thus the prospect of easier and earlier CTEPH diagnosis. In this Series paper, we show how the understanding of CTEPH as a sequela of inflammatory thrombosis has driven successful multidisciplinary management that integrates surgical, interventional, and medical treatments. We provide imaging examples of classical major vessel targets, describe microvascular targets, define available tools, and depict an algorithm facilitating the initial treatment strategy in people with newly diagnosed CTEPH based on a multidisciplinary team discussion at a CTEPH centre. Further work is needed to optimise the use and combination of multimodal therapeutic options in CTEPH to improve long-term outcomes for patients.
Topics: Humans; Artificial Intelligence; Hypertension, Pulmonary; Algorithms; Disease Progression; Inflammation
PubMed: 37591299
DOI: 10.1016/S2213-2600(23)00292-8 -
American Journal of Respiratory and... Aug 2023Pulmonary hypertension, frequently complicating the course of patients with fibrotic interstitial lung disease, is associated with significantly increased morbidity and... (Review)
Review
Pulmonary hypertension, frequently complicating the course of patients with fibrotic interstitial lung disease, is associated with significantly increased morbidity and mortality. The availability of multiple medications to treat pulmonary arterial hypertension has resulted in these agents being used beyond their original indication, including in patients with interstitial lung disease. Whether pulmonary hypertension in the context of interstitial lung disease is an adaptive response not to be treated or a maladaptive phenomenon amenable to therapy has been uncertain. Although some studies have suggested a benefit of treatment, there have been others demonstrating harm. This concise clinical review provides an overview of prior studies and the issues that have plagued drug development for a patient population in dire need of treatment options. More recently, there has been a paradigm shift, with the largest study to date demonstrating benefit, resulting in the first approved therapy in the United States for patients with interstitial lung disease complicated by pulmonary hypertension. A pragmatic management algorithm in the context of changing definitions, comorbid contributors, and an available treatment option is provided, as are considerations for future clinical trials.
Topics: Humans; Familial Primary Pulmonary Hypertension; Hypertension, Pulmonary; Lung Diseases, Interstitial; Pulmonary Arterial Hypertension
PubMed: 37159942
DOI: 10.1164/rccm.202212-2342CI -
European Respiratory Review : An... Sep 2022Pulmonary hypertension (PH) is known to complicate various forms of interstitial lung disease (ILD), including idiopathic pulmonary fibrosis, the interstitial pneumonias... (Review)
Review
Pulmonary hypertension (PH) is known to complicate various forms of interstitial lung disease (ILD), including idiopathic pulmonary fibrosis, the interstitial pneumonias and chronic hypersensitivity pneumonitis. Pathogenesis of PH-ILD remains incompletely understood, and probably has overlap with other forms of pre-capillary pulmonary hypertension. PH-ILD carries a poor prognosis, and is associated with increased oxygen requirements, and a decline in functional capacity and exercise tolerance. Despite most patients having mild-moderate pulmonary hypertension, more severe pulmonary hypertension and signs of right heart failure are observed in a subset of cases. Clinical suspicion and findings on pulmonary function, computed tomography and echocardiography are often the initial steps towards diagnosis. Definitive diagnosis is obtained by right heart catheterisation demonstrating pre-capillary pulmonary hypertension. Drugs approved for pulmonary arterial hypertension have been investigated in several randomised controlled trials in PH-ILD patients, leading to discouraging results until the recent INCREASE study. This review provides an overview of the current understanding, approach to diagnosis and recent advances in treatment.
Topics: Alveolitis, Extrinsic Allergic; Humans; Hypertension, Pulmonary; Idiopathic Pulmonary Fibrosis; Lung; Lung Diseases, Interstitial
PubMed: 35831007
DOI: 10.1183/16000617.0220-2021 -
Clinics in Liver Disease Feb 2023PoPH is a well-recognized complication of portal hypertension with or without cirrhosis and is classified as a subset of PAH. Identification of PoPH is crucial as it has... (Review)
Review
PoPH is a well-recognized complication of portal hypertension with or without cirrhosis and is classified as a subset of PAH. Identification of PoPH is crucial as it has a major impact on prognosis and liver transplant candidacy. Echocardiogram is the initial screening tool of choice and the patient should proceed to RHC for confirmation. PAH-directed therapy is the treatment of choice, allowing the patient to achieve a hemodynamic threshold to undergo a liver transplant safely.
Topics: Humans; Hypertension, Pulmonary; Hypertension, Portal; Liver Transplantation; Prognosis
PubMed: 36400468
DOI: 10.1016/j.cld.2022.08.002 -
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