Pulmonary circulation
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Pulmonary circulation · Apr 2021
Association of daily physical activity with psychosocial aspects and functional capacity in patients with pulmonary arterial hypertension: a cross-sectional study.
Pulmonary arterial hypertension impairs exercise tolerance and daily physical activity. Aside from the hemodynamic limitations, physical, cognitive, and emotional factors may play a relevant and as yet unexplored role. We investigated whether there is an association between the physical activity level and psychological disorders, health-related quality of life, and daily activities. ⋯ Thirty percent scored positive for anxiety, and 15% for depression (Hospital Anxiety and Depression scale). There was a significant correlation between accelerometer data and walking distance (six-minute walk test), number of movements (one-minute sit-to-stand test), level of daily physical activity (Manchester Respiratory Activities of Daily Living questionnaire), and depression symptoms. Our findings support the hypothesis that other aspects beyond physical and hemodynamic ones might impact the daily physical activity of patients with pulmonary arterial hypertension.
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Pulmonary circulation · Jan 2021
Case ReportsRapid dynamic bedside assessment of pulmonary perfusion defect by electrical impedance tomography in a patient with acute massive pulmonary embolism.
Several animal studies have shown that regional lung perfusion could be effectively estimated by the hypertonic saline contrast electrical impedance tomography method. Here, we reported an application of this method to dynamically assess regional pulmonary perfusion defect in a patient with acute massive pulmonary embolism. A 68-year-old man experienced sudden dyspnea and cardiac arrest during out-of-bed physical activity on the first day after partial mediastinal tumor resection. ⋯ Normal ventilation distribution with massive defects in regional perfusion in both lungs was observed, leading to a ventilation-perfusion mismatch and low oxygenation index (PaO2/FiO2 = 86 mmHg) at the first day of pulmonary embolism. The anticoagulation was performed with heparin, and the patient's condition (such as shock, dyspnea, hypoxemia, etc.), regional lung perfusion defect, and ventilation-perfusion mismatch continuously improved in the following days. In conclusion, this case implies that electrical impedance tomography might have the potential to assess and monitor regional perfusion for rapid diagnosis of fatal pulmonary embolism in clinical practice.
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Pulmonary circulation · Oct 2020
Role of biomarkers in evaluation, treatment and clinical studies of pulmonary arterial hypertension.
Pulmonary arterial hypertension is a complex disease resulting from the interplay of myriad biological and environmental processes that lead to remodeling of the pulmonary vasculature with consequent pulmonary hypertension. Despite currently available therapies, there remains significant morbidity and mortality in this disease. There is great interest in identifying and applying biomarkers to help diagnose patients with pulmonary arterial hypertension, inform prognosis, guide therapy, and serve as surrogate endpoints. ⋯ There is growing interest in imaging the lung with various modalities to understand and visualize processes in the lung that lead to pulmonary vascular remodeling including high resolution computed tomography, Xenon magnetic resonance imaging, and positron emission tomography. Such imaging modalities have the potential to demonstrate disease modification resulting from therapeutic interventions. Because right ventricular function is a major determinant of prognosis, imaging of the right ventricle with echocardiography or cardiac magnetic resonance imaging plays an important role in the evaluation of patients and may also be useful in clinical studies of pulmonary arterial hypertension.
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Pulmonary circulation · Jul 2020
Case ReportsA novel BMPR2 mutation with widely disparate heritable pulmonary arterial hypertension clinical phenotype.
Mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2) have been associated with heritable pulmonary arterial hypertension (HPAH), whereas mutations in the gene encoding eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) are associated with heritable pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (HPVOD/PCH). We describe two unrelated patients found to carry the same hitherto unreported pathogenic BMPR2 mutation; one of whom presented with typical pulmonary arterial hypertension, whereas the second patient presented with aggressive disease and characteristic clinical features of PVOD/PCH. These two clinically divergent cases representative of the same novel pathogenic mutation exemplify the variable phenotype of HPAH and the variable involvement of venules and capillaries in the pathology of the pulmonary vascular bed in pulmonary arterial hypertension.
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Pulmonary circulation · Jul 2020
Current clinical utilization of risk assessment tools in pulmonary arterial hypertension: a descriptive survey of facilitation strategies, patterns, and barriers to use in the United States.
Practice guidelines suggest that treatment decisions in pulmonary arterial hypertension be informed by periodic assessment of patients' clinical risk. Several tools, well validated for risk discrimination, such as the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management calculator, were developed to assess pulmonary arterial hypertension patients' risk of death based on multiple parameters, including functional class, hemodynamics, biomarkers, comorbidities, and exercise capacity. Using an online survey, we investigated the use of risk assessment tools by pulmonary hypertension healthcare providers in the United States. ⋯ Risk was most frequently assessed by decision makers at the time of diagnosis (cited by 54%) and at the time of worsening symptoms (cited by 42%), suggesting that use of pulmonary arterial hypertension risk assessment tools remains low. In our survey, non-physicians compared with physicians cited two major barriers to increased tool use: lack of education and training (20% vs. 4%) and lack of clarity on the best tool to use (30% vs. 18%). Information technology tools, such as electronic medical record integration and web or phone-based risk calculating applications, were cited most frequently as ways to increase the use of risk assessment tools.