Pulmonary veno-occlusive disease: a probably underdiagnosed cause of pulmonary hypertension in systemic sclerosis

We report the case of a 72-year-old woman with limited systemic sclerosis, who was initially diagnosed with systemic sclerosis-related pulmonary arterial hypertension. However, after introduction of sildenafil and bosentan, the patient developed acute pulmonary oedema, and findings from complementary exams were suggestive of PVOD.
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research

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Conclusions: Psychological evaluation and social support become mandatory because their prognosis depends on these issues.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Background: Patients with systemic sclerosis (SSc) are at high risk of developing pulmonary hypertension (PH) which crucially affects patients’ symptoms, quality of life and prognosis. The aim of this study was to analyse the impact of the new hemodynamic definition of precapillary PH as proposed at the 2018 World Symposium on PH in SSc-patients.Methods: SSc-patients were screened for PH using clinical assessments as WHO-functional class, 6 minute walking distance (6MWD) and right heart catheterisation. Patients were divided into hemodynamic subgroups according to their mean pulmonary arterial pressure (mPAP) values ...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
ConclusionsPrevalence of PAH was lower than expected (3.7%). Probability of having elevated esPAP was higher among patients with dSSc and among those with ATA.
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
Pulmonary arterial hypertension (PAH) is a life-shortening complication of systemic sclerosis (SSc) with a life-time prevalence of approximately 10% [1, 2]. It is currently defined by the presence of a mean pulmonary arterial pressure (mPAP) ≥25 mmHg with a pulmonary arterial wedge pressure (PAWP) ≤15 mmHg and a pulmonary vascular resistance (PVR)>3 WU, in the absence of significant lung disease or chronic thromboembolic disease. Other forms of pulmonary hypertension (PH) may also quite commonly exist in SSc patients, including PH due to left heart disease or lung disease. It is widely agreed that i...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Editorials Source Type: research
e E Abstract Exercise stress testing of the pulmonary circulation for the diagnosis of latent or early stage pulmonary hypertension is gaining acceptance. There is emerging consensus to define exercise-induced pulmonary hypertension by a mean pulmonary artery pressure higher than 30 mmHg at a cardiac output of less than 10 L/min and a total pulmonary vascular resistance higher than 3 Wood units at maximum exercise, in the absence of pulmonary hypertension at rest. Exercise-induced pulmonary hypertension has been reported in association with a bone morphogenetic receptor-2 gene mutation, in systemic sclerosis, in l...
Source: Chest - Category: Respiratory Medicine Authors: Tags: Chest Source Type: research
Conclusion: CC was frequent among patients with SSc-ILD but was not associated with increased mortality. As expected, PH was associated with severe excess mortality.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Diffuse Parenchymal Lung Disease Source Type: research
Pulmonary hypertension (PH) is a frequent and severe complication of systemic sclerosis (SSc). PH in SSc is highly heterogeneous because of the various clinical phenotypes of SSc itself and because the mechanisms of PH can vary from one patient to another. PH in SSc may be due to vasculopathy of the small pulmonary arteries (group 1; pulmonary arterial hypertension), interstitial lung disease (group 3; PH due to lung disease or chronic hypoxia) or myocardial fibrosis leading to left ventricular systolic or diastolic dysfunction (group 2; PH due to chronic left-heart disease). Pulmonary veno-occlusive disease is not uncommo...
Source: European Respiratory Review - Category: Respiratory Medicine Authors: Tags: Interstitial and orphan lung disease, Pulmonary vascular disease Reviews Source Type: research
Abstract BACKGROUND: Previous studies have identified survival in systemic sclerosis (SSc)-associated pulmonary arterial hypertension (SSc-PAH) to be worse than in idiopathic pulmonary arterial hypertension (IPAH). We investigated differences between these conditions by comparing demographic, haemodynamic and radiological characteristics and outcomes in a large cohort of incident patients. METHODS: 651 patients diagnosed with IPAH or SSc-associated pre-capillary pulmonary hypertension were included. Patients with lung disease or ≥2 risk factors for left heart disease were identified leaving a primary analy...
Source: Chest - Category: Respiratory Medicine Authors: Tags: Chest Source Type: research
Introduction and classification Pulmonary hypertension (PH) encompasses a group of diseases associated with an elevated mean pulmonary artery pressure (mPAP) ≥25 mm Hg at rest. The symptoms of PH are non-specific, and it should be suspected in any patient with unexplained exertional dyspnoea, exertional dizziness (near syncope), syncopal episodes and/or signs of right ventricular dysfunction. PH can predominantly affect the precapillary part of the pulmonary circulation, for example, pulmonary arterial hypertension (PAH) or postcapillary—pulmonary venous circulation due to left heart disease (PH-LHD). A ...
Source: Heart - Category: Cardiology Authors: Tags: Education in Heart, Congenital heart disease, Drugs: cardiovascular system, Hypertension, Epidemiology, Metabolic disorders Source Type: research
We included 59 PAH pts withangina-like symptoms: 28 pts (25 f/3m) with IPAH, 18 pts (14 f/4m) with congenital heart disease and 13 pts (12 f/1m) PAH associated with systemic scleroderma (SS), of comparable age: 36,86±11,5/44±17,47/46,32±15,71yrs respectively. On the 1st step of diagnostic examination we found the pts with ischemic changes on Holter ECG. In all pts with the identification of ischemic changes were performed single photon emission CT with 99mTc-MIBI myocardium at rest and on the peak of stress test.By Holter we identified the next change: in IPAH/PAH-CHD/PAH-SS group mean HR were 73,45&pl...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: 4.3 Pulmonary Circulation and Pulmonary Vascular Disease Source Type: research
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