21 Result(s)
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Article
Open AccessHemodynamic and clinical onset in patients with hereditary pulmonary arterial hypertension and BMPR2 mutations
Mutations in the bone morphogenetic protein receptor 2 (BMPR2) gene can lead to idiopathic pulmonary arterial hypertension (IPAH). This study prospectively screened for BMPR2 mutations in a large cohort of PAH-pa...
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Open AccessHemodynamic and genetic analysis in children with idiopathic, heritable, and congenital heart disease associated pulmonary arterial hypertension
Aim of this prospective study was to compare clinical and genetic findings in children with idiopathic or heritable pulmonary arterial hypertension (I/HPAH) with children affected with congenital heart defects...
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Article
Exercise Capacity Affects Quality of Life in Patients with Pulmonary Hypertension
The objective of this prospective study was to evaluate the impact of exercise capacity, mental disorders, and hemodynamics on quality-of-life (QoL) parameters in patients with pulmonary arterial hypertension ...
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Open AccessAnxiety and depression disorders in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension
The objective of this prospective study was to assess the prevalence of anxiety and depression disorders and their association with quality of life (QoL), clinical parameters and survival in patients with pulm...
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Article
The Combination of Exercise and Respiratory Training Improves Respiratory Muscle Function in Pulmonary Hypertension
Increased dyspnea and reduced exercise capacity in pulmonary arterial hypertension (PAH) can be partly attributed to impaired respiratory muscle function. This prospective study was designed to assess the impa...
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Article
Economic Evaluation of Exercise Training in Patients with Pulmonary Hypertension
Exercise training as an add-on to medical therapy has been shown to improve exercise capacity, quality of life, and possibly prognosis in patients with pulmonary hypertension (PH). The purpose of this study wa...
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Article
Safety and Long-Term Efficacy of Transition from Sildenafil to Tadalafil due to Side Effects in Patients with Pulmonary Arterial Hypertension
Two phosphodiesterase-type 5 (PDE-5) inhibitors, sildenafil and tadalafil, are approved for treatment of pulmonary arterial hypertension (PAH). It has not yet been observed if transition from sildenafil to tad...
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MR-proADM Predicts Exercise Capacity and Survival Superior to Other Biomarkers in PH
Besides the established biomarker NT-proBNP, the new cardiovascular biomarkers MR-proANP, MR-proADM, Copeptin, and CT-proET-1 are promising to evaluate hemodynamics, exercise parameters, and prognosis in pati...
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Article
Open AccessEIF2AK4 mutation as “second hit” in hereditary pulmonary arterial hypertension
Mutations in the eukaryotic translation initiation factor 2α kinase 4 (EIF2AK4) gene have recently been identified in recessively inherited veno-occlusive disease. In this study we assessed if EIF2AK4 mutations o...
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Article
Open AccessAcute effects of exercise on the inflammatory state in patients with idiopathic pulmonary arterial hypertension
Exercise training positively influences exercise tolerance and functional capacity of patients with idiopathic pulmonary arterial hypertension (IPAH). However, the underlying mechanisms are unclear. We hypothe...
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Open AccessRiociguat in patients with chronic thromboembolic pulmonary hypertension: results from an early access study
Following positive results from the Phase III CHEST-1 study in patients with inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH), the Phase IIIb CTEPH early access study (E...
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Correction to: Real-World Switching to Riociguat: Management and Practicalities in Patients with PAH and CTEPH
The original version of this article unfortunately contained a mistake. In the “Results” section, the percentage of patients with inoperable or persistent/recurrent CTEPH included in the study was reported as ...
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Article
Open AccessReal-World Switching to Riociguat: Management and Practicalities in Patients with PAH and CTEPH
A proportion of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) do not achieve treatment goals or experience side effects on their current therapy....
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Open AccessRight heart size and function significantly correlate in patients with pulmonary arterial hypertension – a cross-sectional study
The objective of this study was to assess, whether right atrial (RA) and ventricular (RV) size is related to RV pump function at rest and during exercise in patients with pulmonary arterial hypertension (PAH).
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Article
Open AccessRight ventricular size and function under riociguat in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (the RIVER study)
Riociguat is a soluble guanylate cyclase stimulator approved for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTPEH). The objective of this study was to evaluate rig...
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Open AccessClinical outcomes stratified by baseline functional class after initial combination therapy for pulmonary arterial hypertension
Initial combination therapy with ambrisentan and tadalafil reduced the risk of clinical failure events for treatment-naïve participants with pulmonary arterial hypertension (PAH) as compared to monotherapy. Pr...
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Article
Open AccessRisk stratification and prognostic factors in patients with pulmonary arterial hypertension and comorbidities a cross-sectional cohort study with survival follow-up
The objective of this study was to analyze prognostic factors and risk stratification in patients with pulmonary arterial hypertension (PAH) and comorbidities.
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Article
Open AccessPrognostic impact of hypochromic erythrocytes in patients with pulmonary arterial hypertension
Iron deficiency affects up to 50% of patients with pulmonary arterial hypertension (PAH) but iron markers such as ferritin and serum iron are confounded by several non-disease related factors like acute inflam...
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Article
Open AccessGene panel diagnostics reveals new pathogenic variants in pulmonary arterial hypertension
A genetic predisposition can lead to the rare disease pulmonary arterial hypertension (PAH). Most mutations have been identified in the gene BMPR2 in heritable PAH. However, as of today 15 further PAH genes have ...
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Article
Open AccessTolerability, safety and survival in patients with severe pulmonary arterial hypertension treated with intravenous epoprostenol (Veletri®): a prospective, 6-months, open label, observational, non-interventional study
Epoprostenol AS (Veletri®), a thermostable epoprostenol formulation, provides better drug stability and improved clinical use compared to previous epoprostenol formulations. This study aims to expand clinical exp...
