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Article
Primary Pulmonary Hypertension in Children May Have a Different Genetic Background Than in Adults
Mutations of the bone morphogenetic protein receptor II (BMPR2) gene on chromosome 2q33 can cause familial primary pulmonary hypertension (PPH) and may occur in 26% adult patients with sporadic disease. Other dis...
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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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Article
Open AccessExercise training in pulmonary arterial hypertension associated with connective tissue diseases
The objective of this prospective study was to assess short- and long-term efficacy of exercise training (ET) as add-on to medical therapy in patients with connective tissue disease-associated pulmonary arteri...
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Article
Open AccessThe German adaptation of the Cambridge pulmonary hypertension outcome review (CAMPHOR)
Individuals with precapillary pulmonary hypertension (PH) experience severely impaired quality of life. A disease-specific outcome measure for PH, the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) ...
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Article
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
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
Open AccessBorderline pulmonary arterial pressure in systemic sclerosis patients: a post-hoc analysis of the DETECT study
Patients with mean pulmonary artery pressures (mPAP) of 21 to 24 mm Hg have a so-called borderline elevation of mPAP (BoPAP)—a condition thought to represent early-stage pulmonary arterial vasculopathy. Based ...
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Article
Open AccessStress Doppler echocardiography for early detection of systemic sclerosis-associated pulmonary arterial hypertension
In patients with systemic sclerosis (SSc), associated pulmonary arterial hypertension (SSc-APAH) is the leading cause of death. The objective of this prospective screening study was to analyse sensitivity and ...
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Article
Open AccessLate outcomes after acute pulmonary embolism: rationale and design of FOCUS, a prospective observational multicenter cohort study
Acute pulmonary embolism (PE) is a frequent cause of death and serious disability. The risk of PE-associated mortality and morbidity extends far beyond the acute phase of the disease. In earlier follow-up stud...
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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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Article
Open AccessAbstracts from the 8th International Conference on cGMP Generators, Effectors and Therapeutic Implications
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Article
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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Article
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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Article
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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Article
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 AccessEarly treatment with ambrisentan of mildly elevated mean pulmonary arterial pressure associated with systemic sclerosis: a randomized, controlled, double-blind, parallel group study (EDITA study)
The objective of this randomized, placebo-controlled, double-blind, parallel group, trial was to assess the effect of ambrisentan on mean pulmonary arterial pressure (mPAP) in patients with systemic sclerosis ...
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Article
Open AccessTherapeutic potential of KLF2-induced exosomal microRNAs in pulmonary hypertension
Pulmonary arterial hypertension (PAH) is a severe disorder of lung vasculature that causes right heart failure. Homoeostatic effects of flow-activated transcription factor Krüppel-like factor 2 (KLF2) are comp...
