scholarly journals Pulmonary hypertension in children with chronic bronchopulmonary pathology: a pulmonologist’s perspective to the problem

2021 ◽  
pp. 300-309
Author(s):  
A. R. Shudueva ◽  
Yu. L. Mizernitskiy ◽  
O. S. Groznova ◽  
L. I. Agapitov
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Diseases ◽  
Young Patients ◽  
Cor Pulmonale ◽  
Lung Pathology ◽  
Chronic Lung Diseases ◽  
Pulmonary Hemodynamic ◽  
Depth Study ◽  
Special Approach ◽  
Hypertension In Children

The respiratory diseases are consistently ranked first in the morbidity pattern among children and adolescents in the Russian Federation. Registry data show that 10-12% of children with pulmonary hypertension have PH-associated chronic lung pathology. Pulmonary hypertension as a life-threatening complication of such chronic lung diseases as hypersensitive pneumonitis, bronchiectasis, bronchial asthma, etc. aggravates the severity of their clinical course and has a great impact on the outcome of the disease. The article summarizes modern data on pulmonary hypertension in children of various ages associated with chronic bronchopulmonary pathology. The issues of classification, pathogenetic mechanisms, criteria and modern methods of diagnosis and treatment, as well as prognosis are considered. The authors come to the conclusion that despite the in-depth study of the pathogenetic aspects of the development of pulmonary hypertension in chronic bronchopulmonary pathology in children, which has recently made it possible to shed light on the understanding of many of its aspects, it should be admitted that the problem as a whole is far from being resolved. Early detection and treatment of pulmonary hemodynamic pathology is extremely important to prevent the formation of cor pulmonale. Pulmonary hypertension, which complicates the chronic lung diseases, is still incurable and is associated with high mortality. Now, young patients with pulmonary hypertension are still often examined and treated using guidelines for adult patients. However, differences in etiology, clinical presentations and diagnostic capabilities require a special approach to studying this problem in children, which raises the question of the need to develop separate clinical guidelines for pediatric practice.

scholarly journals Prescription Patterns for Pulmonary Vasodilators in the Treatment of Pulmonary Hypertension Associated With Chronic Lung Diseases: Insights From a Clinician Survey

2021 ◽  
Vol 8 ◽  
Author(s):  
Christopher A. Thomas ◽  
Justin Lee ◽  
Roberto J. Bernardo ◽  
Ryan J. Anderson ◽  
Vladimir Glinskii ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Targeted Therapy ◽  
Lung Disease ◽  
Lung Diseases ◽  
Online Survey ◽  
Treatment Approach ◽  
Initial Therapy ◽  
Triple Combination Therapy ◽  
Management Guidelines ◽  
Chronic Lung Diseases

Background: Pulmonary hypertension is a complication of chronic lung diseases (PH-CLD) associated with significant morbidity and mortality. Management guidelines for PH-CLD emphasize the treatment of the underlying lung disease, but the role of PH-targeted therapy remains controversial. We hypothesized that treatment approaches for PH-CLD would be variable across physicians depending on the type of CLD and the severity of PH.Methods and Results: Between May and July 2020, we conducted an online survey of PH experts asking for their preferred treatment approach in seven hypothetical cases of PH-CLD of varying severity. We assessed agreement amongst clinicians for initial therapy choice using Fleiss' kappa calculations. Over 90% of respondents agreed that they would treat cases of severe PH in the context of mild lung disease with some form of PH-targeted therapy. For cases of severe PH in the context of severe lung disease, over 70% of respondents agreed to use PH-targeted therapy. For mild PH and mild lung disease cases, <50% of respondents chose to start PH-specific therapy. There was overall poor agreement between respondents in the choice to use mono-, double or triple combination therapy with PH-specific agents in all cases.Conclusion: Although management guidelines discourage the routine use of PH-targeted therapies to treat PH-CLD patients, most physicians choose to treat patients with some form of PH-targeted therapy. The choice of therapy and treatment approach are variable and appear to be influenced by the severity of the PH and the underlying lung disease.

scholarly journals Pulmonary Hypertension in Chronic Lung Diseases and/or Hypoxia

10.5772/55681 ◽  
2013 ◽  
Author(s):  
Dimitar Sajkov ◽  
Bliegh Mupunga ◽  
Jeffrey J. ◽  
Nikolai Petrovsky
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Diseases ◽  
Chronic Lung Diseases

scholarly journals Macrophage bone morphogenic protein receptor 2 depletion in idiopathic pulmonary fibrosis and Group III pulmonary hypertension

2016 ◽  
Vol 311 (2) ◽  
pp. L238-L254 ◽  
Author(s):  
Ning-Yuan Chen ◽  
Scott D. Collum ◽  
Fayong Luo ◽  
Tingting Weng ◽  
Thuy-Trahn Le ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Diseases ◽  
Vascular Lesions ◽  
Unknown Etiology ◽  
Group Iii ◽  
Chronic Lung Diseases ◽  
Protein Receptor ◽  
Stat3 Phosphorylation

Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease of unknown etiology. The development of pulmonary hypertension (PH) is considered the single most significant predictor of mortality in patients with chronic lung diseases. The processes that govern the progression and development of fibroproliferative and vascular lesions in IPF are not fully understood. Using human lung explant samples from patients with IPF with or without a diagnosis of PH as well as normal control tissue, we report reduced BMPR2 expression in patients with IPF or IPF+PH. These changes were consistent with dampened P-SMAD 1/5/8 and elevated P-SMAD 2/3, demonstrating reduced BMPR2 signaling and elevated TGF-β activity in IPF. In the bleomycin (BLM) model of lung fibrosis and PH, we also report decreased BMPR2 expression compared with control animals that correlated with vascular remodeling and PH. We show that genetic abrogation or pharmacological inhibition of interleukin-6 leads to diminished markers of fibrosis and PH consistent with elevated levels of BMPR2 and reduced levels of a collection of microRNAs (miRs) that are able to degrade BMPR2. We also demonstrate that isolated bone marrow-derived macrophages from BLM-exposed mice show reduced BMPR2 levels upon exposure with IL6 or the IL6+IL6R complex that are consistent with immunohistochemistry showing reduced BMPR2 in CD206 expressing macrophages from lung sections from IPF and IPF+PH patients. In conclusion, our data suggest that depletion of BMPR2 mediated by a collection of miRs induced by IL6 and subsequent STAT3 phosphorylation as a novel mechanism participating to fibroproliferative and vascular injuries in IPF.

scholarly journals Pulmonary Hypertension: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases

2014 ◽  
Vol 11 (Supplement 3) ◽  
pp. S178-S185 ◽  
Author(s):  
Eric D. Austin ◽  
Steven M. Kawut ◽  
Mark T. Gladwin ◽  
Steven H. Abman
Keyword(s):  
Pulmonary Hypertension ◽  
Primary Prevention ◽  
Lung Diseases ◽  
Chronic Lung Diseases

scholarly journals Right ventricular diastolic function in patients with lung diseases in the absence or presence of pulmonary hypertension

2017 ◽  
Vol 89 (3) ◽  
pp. 54-60 ◽  
Author(s):  
A N Sumin ◽  
O G Arkhipov
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Diseases ◽  
Tissue Doppler ◽  
Doppler Imaging ◽  
Chronic Lung Diseases ◽  
Respiratory Pathology ◽  
Right Ventricular Diastolic Function ◽  
Flow Propagation Velocity ◽  
The Right ◽  
Flow Propagation

Aim. To investigate the indicators of filling of the right ventricle (RV) in patients with chronic lung diseases with and without pulmonary hypertension (PH) compared to healthy individuals. Subjects and methods. 365 people (198 men); mean age 64.6±8.0 years) were examined and divided into a group of patients with respiratory pathology without and with PH (n=124 and n=138, respectively) and a comparison group that included individuals without cardiovascular and respiratory diseases (n=103). All underwent echocardiography with examination of RV filling flows (Et, At, Et/At), data of the spectral tissue Doppler imaging of the fibrous ring of the tricuspid valve (e’t, a’t, e’t/a’t), and early tricuspid flow propagation velocity (ETFPV). Results. All the groups were found to have an e’t/a’t decrease to 0.75 (0.63—0.90) — 0.8 (0.63—1.0; p=0.26). The groups showed no noticeable differences in indicators, such as Еt/Аt, e’t/a’t, and Еt/e’t ratios, although the increased size of the right heart was noted in patients without PH along with the ETFPV decrease from 33.5 (28—39) to 31.5 (24.5—36) cm/sec, which continued to substantially decline to 27.1 (24—35) cm/sec in patients with PH (p

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