Relationship between time from diagnosis and morbidity/mortality in pulmonary arterial hypertension: results from the phase III GRIPHON study.

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Early initiation of pulmonary arterial hypertension (PAH) therapies is associated with improved long-term outcomes, yet data on the early use of prostacyclin pathway agents are limited. In these post hoc […]

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Imaging of pulmonary hypertension in adults: a position paper from the Fleischner Society.

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Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure greater than 20 mmHg and classified into five different groups sharing similar pathophysiologic mechanisms, haemodynamic characteristics, and therapeutic management. Radiologists […]

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Screening for pulmonary arterial hypertension in adults carrying a BMPR2 mutation.

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Heritable pulmonary arterial hypertension (PAH) is most commonly due to heterozygous mutations of the BMPR2 gene. Based on expert consensus, guidelines recommend annual screening echocardiography in asymptomatic BMPR2 mutation carriers. […]

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ERS Statement on Chronic Thromboembolic Pulmonary Hypertension.

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Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, either symptomatic or not. The occlusion of proximal pulmonary arteries by fibrotic intravascular material, in combination with […]

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ERS Statement on Chronic Thromboembolic Pulmonary Hypertension.

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Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, either symptomatic or not. The occlusion of proximal pulmonary arteries by fibrotic intravascular material, in combination with […]

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Prolonged Elevation of Tricuspid Regurgitation Pressure Gradient after Exercise in Patients with Exercise-Induced Pulmonary Hypertension.

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It is necessary to measure the peak tricuspid regurgitation pressure gradient (TRPG) that is recorded at maximum exercise intensity when diagnosing exercise-induced pulmonary hypertension (ePH) on exercise stress echocardiography (ESE). […]

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EmPHasis-10 as a Measure of Health-Related Quality of Life in Pulmonary Arterial Hypertension: data from PHAR.

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While the performance of the emPHasis-10 (e10) score has been evaluated against limited patient characteristics within the United Kingdom, there is an unmet need for exploring the performance of the […]

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Disproportionate secondary mitral regurgitation: myths, misconceptions and clinical implications.

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Secondary (functional) mitral regurgitation (SMR) most commonly arises secondary to left ventricular (LV) dilation/dysfunction. The concept of disproportionately severe SMR was proposed to help explain the different results of two […]

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Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial.

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This prospective, randomized, controlled, multicentre study aimed to evaluate efficacy and safety of exercise training in patients with pulmonary arterial (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH).For the first time […]

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Plasma metabolomics exhibit response to therapy in chronic thromboembolic pulmonary hypertension.

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Pulmonary hypertension is a condition with limited effective treatment options. Chronic thromboembolic pulmonary hypertension (CTEPH) is a notable exception with pulmonary endarterectomy (PEA) often proving curative. This study investigated the […]

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Renin-Angiotensin-Aldosterone System Inhibitor Use and Mortality in Pulmonary Hypertension: Insights from the Veterans Affairs CART Database.

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The renin-angiotensin-aldosterone system (RAAS) contributes to pulmonary hypertension (PH) pathogenesis. While animal data suggest RAAS inhibition attenuates PH, it is unknown if RAAS inhibition is beneficial in PH patients.Is RAAS […]

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Prevalence of pulmonary hypertension in adults after atrial switch and role of ventricular filling pressures.

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To assess the prevalence of elevated systemic right ventricular (sRV) end-diastolic pressure and pulmonary arterial hypertension in adults with transposition of the great arteries (TGA) who have undergone atrial switch […]

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Clinical and echocardiographic outcomes in heart failure associated with methamphetamine use and cessation.

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Methamphetamine use is associated with systolic dysfunction, pulmonary arterial hypertension and may also be associated with diastolic dysfunction. The impact of methamphetamine cessation on methamphetamine-associated heart failure (MethHF) remains poorly […]

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Characteristics and long-term outcomes of pulmonary veno-occlusive disease induced by Mitomycin-C.

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Pulmonary veno-occlusive disease (PVOD) is an uncommon form of pulmonary hypertension (PH) predominantly characterized by pulmonary vein and capillary involvement. An association between chemotherapy, in particular mitomycin-C (MMC), and PVOD […]

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Targeting HIF2α-ARNT hetero-dimerisation as a novel therapeutic strategy for Pulmonary Arterial Hypertension.

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Pulmonary Arterial Hypertension (PAH) is a destructive disease of the pulmonary vasculature often leading to right heart failure and death. Current therapeutic intervention strategies only slow disease progression. The role […]

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Influence of Body Weight and Diabetes Mellitus in Patients With Pulmonary Hypertension.

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Pulmonary hypertension (PH) is a complex condition that arises due to pulmonary vascular disease, heart disease, lung disease, chronic thromboembolism, or several rare causes. Regardless of underlying cause, PH increases […]

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Timing of intervention in asymptomatic patients with valvular heart disease.

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Current management of valvular heart disease (VHD) seeks to optimize long-term outcome by timely intervention. Recommendations for treatment of patients with symptoms due to severe valvular disease are based on […]

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Right ventricle in heart failure with preserved ejection fraction.

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Heart failure with preserved ejection fraction (HFpEF) affects half of all patients with heart failure. While previously neglected, the right ventricle (RV) has sparked interest in recent years as a […]

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Prognostic Implications of Significant Isolated Tricuspid Regurgitation in Patients With Atrial Fibrillation Without Left-Sided Heart Disease or Pulmonary Hypertension.

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The prognostic impact of isolated tricuspid regurgitation (TR) in patients with atrial fibrillation (AF) has not been investigated. The purpose of this study was to investigate the prognostic implications of […]

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Efficacy and safety of sildenafil added to pirfenidone in patients with advanced idiopathic pulmonary fibrosis and risk of pulmonary hypertension: a double-blind, randomised, placebo-controlled, phase 2b trial.

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The benefit of sildenafil in patients with advanced idiopathic pulmonary fibrosis (IPF) at risk of poor outcomes from pulmonary hypertension, whether already present or likely to develop, is uncertain. We […]

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Targeting Transforming Growth Factor Beta Receptors in Pulmonary Hypertension.

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The transforming growth factor (TGF)-β superfamily includes several groups of multifunctional proteins that form two major branches, namely the TGF-β/activin/nodal branch and the bone morphogenetic protein (BMP)/growth differentiation factor (GDF) […]

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Sleep-related breathing disorders and pulmonary hypertension.

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Sleep-related breathing disorders include obstructive apnoea, central apnoea and sleep-related hypoventilation. These nocturnal events have the potential to increase pulmonary arterial pressure during sleep but also in the waking state. […]

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