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  1. 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...

    Ekkehard Grünig, Rolf Koehler, Gabriel Miltenberger-Miltenyi in Pediatric Research (2004)

  2. No Access

    Article

    Value of high spatial and high temporal resolution magnetic resonance angiography for differentiation between idiopathic and thromboembolic pulmonary hypertension: initial results

    Differentiation between different forms of pulmonary hypertension (PH) is essential for correct disease management. The goal of this study was to elucidate the clinical impact of high spatial resolution MR ang...

    Sebastian Ley, Christian Fink, Julia Zaporozhan, Mathias M. Borst in European Radiology (2005)

  3. Article

    Open Access

    Hemodynamic 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...

    Nicole Pfarr, Justyna Szamalek-Hoegel, Christine Fischer in Respiratory Research (2011)

  4. Article

    Open Access

    Exercise 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...

    Ekkehard Grünig, Felicitas Maier, Nicola Ehlken in Arthritis Research & Therapy (2012)

  5. Article

    Open Access

    The 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) ...

    Katharina Cima, James Twiss, Rudolf Speich in Health and Quality of Life Outcomes (2012)

  6. Article

    Open Access

    Hemodynamic 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...

    Nicole Pfarr, Christine Fischer, Nicola Ehlken, Tabea Becker-Grünig in Respiratory Research (2013)

  7. No Access

    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 ...

    Michael Halank, Franziska Einsle, Stephanie Lehman, Hinrich Bremer, Ralf Ewert in Lung (2013)

  8. Article

    Open Access

    Anxiety 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...

    Dominik Harzheim, Hans Klose, Fabiola Peña Pinado, Nicola Ehlken in Respiratory Research (2013)

  9. No Access

    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...

    Hans-Joachim Kabitz, Hinrich-Cordt Bremer, Anja Schwoerer, Florian Sonntag in Lung (2014)

  10. No Access

    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...

    Nicola Ehlken, Cora Verduyn, Henning Tiede, Gerd Staehler, Gabriele Karger in Lung (2014)

  11. Article

    Open Access

    Borderline 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 ...

    Scott H Visovatti, Oliver Distler, J Gerry Coghlan in Arthritis Research & Therapy (2014)

  12. No Access

    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...

    Mona Lichtblau, Dominik Harzheim, Nicola Ehlken, Alberto Marra, Fabiola Pena Pinado in Lung (2015)

  13. Article

    Open Access

    Stress 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 ...

    Christian Nagel, Philipp Henn, Nicola Ehlken in Arthritis Research & Therapy (2015)

  14. No Access

    Article

    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...

    Martin Kolditz, Hans-Jürgen Seyfarth, Heinrike Wilkens, Ralf Ewert, Tom Bollmann in Lung (2015)

  15. No Access

    Article

    Right atrial morphology and function in patients with systemic sclerosis compared to healthy controls: a two-dimensional strain study

    Enlargement and dysfunction of the right atrium might be an early sign for pulmonary hypertension in systemic sclerosis (SSc). This is the first study to analyse right atrial morphology and function in SSc pat...

    Antonello D’Andrea, Michele D’Alto, Marco Di Maio, Serena Vettori in Clinical Rheumatology (2016)

  16. Article

    Open Access

    Late 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...

    Stavros V. Konstantinides, Stefano Barco in Journal of Thrombosis and Thrombolysis (2016)

  17. Article

    Open Access

    EIF2AK4 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...

    Christina A. Eichstaedt, Jie Song, Nicola Benjamin in Respiratory Research (2016)

  18. Article

    Open Access

    Acute 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...

    Lars Harbaum, Emilia Renk, Sara Yousef, Antonia Glatzel in BMC Pulmonary Medicine (2016)

  19. No Access

    Article

    Reference ranges and determinants of right ventricle outflow tract acceleration time in healthy adults by two-dimensional echocardiography

    The right ventricular outflow tract acceleration time (RVOT-AT) has shown to progressively shorten with increasing degrees of pulmonary pressure. However, the physiologic ranges of RVOT AT are based on small s...

    Alberto M. Marra, Nicola Benjamin in The International Journal of Cardiovascula… (2017)

  20. No Access

    Article

    Intravenous treprostinil infusion via a fully implantable pump for pulmonary arterial hypertension

    Parenteral prostanoids infused via external pumps are well-established pulmonary arterial hypertension (PAH) treatments. However, local side-effects and systemic infections restrict their use. The purpose of t...

    Ralf Ewert, Manuel J. Richter in Clinical Research in Cardiology (2017)

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