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Background: The coupling of the right ventricle (RV) to the pulmonary circulation is an indicator of RV performance that can be non-invasively estimated by echocardiography. There are no data about its use in patients affected by fibrotic interstitial lung diseases (f-ILD). Methods: Fifty f-ILD patients, including 27 cases with idiopathic pulmonary fibrosis (IPF) (M = 37; mean age 67 ± 7 years), were studied with standard and speckle-tracking echocardiography and compared with 30 age-matched healthy volunteers. The mean patient follow-up was 70 ± 4 months. Results: Fibrotic ILD patients had a larger right ventricle (RV) and worse diastolic function because the RV global longitudinal strain (GLS) was significantly lower and the systolic pulmonary artery pressure (sPAP) estimates were higher in comparison with those of controls. Conversely, tricuspid annular systolic excursion (TAPSE) did not differ between controls and patients. Median values of TAPSE/sPAP and RV GLS/sPAP were significantly reduced in f-ILD patients (p < 0.0001). Patients with an RV GLS/sPAP below the median value had a shorter survival time (61 vs. 74 months, p = 0.01); this parameter was an independent predictor of a worse outcome. Conclusion: Low estimates of RV GLS/sPAP are predictive of worse outcomes in f-ILD patients. RV coupling seems to be a promising surrogate biomarker of RV performance to discriminate the patient phenotype with significant management and prognosis implications.
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http://dx.doi.org/10.3390/jcm11206115 | DOI Listing |
BMC Pulm Med
August 2025
Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No.1 Shuaifuyuan St, Dongcheng District, Beijing, 100730, China.
Introduction: Telomere and telomerase abnormalities play critical roles in interstitial lung diseases (ILDs). This study aimed to explore the telomere lengths (TL) in cells in the peripheral blood and bronchoalveolar lavage fluid (BALF) of healthy individuals and patient with various types of non-idiopathic pulmonary fibrosis (IPF)-ILD and to evaluate the correlation between TL and clinical indicators.
Methods: We enrolled 48 patients with ILDs and 21 control individuals who presented at our hospital from September 2023 to September 2024.
Expert Rev Respir Med
July 2025
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
Introduction: The fibrotic interstitial lung diseases ;(f-ILD) are often progressive and debilitating lung diseases with significant symptom burden and impaired quality of life. Pulmonary rehabilitation (PR) has demonstrated improvements in dyspnea, physical function, and respiratory-related quality of life (RR-QoL) in patients with -f-ILD but had limited uptake and completion in real-world settings.
Areas Covered: A literature review was completed on the impact and limitations of traditional PR in f-ILD, with additional perspective on the use of home-based approaches and implementation of health coaching.
Respirology
June 2025
Integrated Care Center of Interstitial Lung Disease, Taichung Veterans General Hospital, Taichung, Taiwan.
Background And Objective: Fibrotic interstitial lung disease (F-ILD) has high mortality, necessitating multidimensional staging to assess severity, predict prognosis, and guide treatment. However, the gender-age-physiology (GAP) index lacks patient-reported outcome measures (PROMs) and exercise test parameters, and traditional methods may miss complex variable interactions. The study aimed to develop a risk model of mortality in F-ILD using routine PROMs and exercise test parameters, and to compare the predictive performance with GAP staging system.
View Article and Find Full Text PDFExpert Rev Respir Med
September 2025
Faculty of Hamidiye Health Sciences, Department of Physiotherapy and Rehabilitation, Gedik University, Istanbul, Turkey.
Background: Fibrosing Interstitial Lung Diseases (F-ILDs) lead to reduced exercise capacity and quality of life. Pulmonary Rehabilitation (PR) exercise programs have shown potential in improving symptoms and functional capacity in these patients. This study aimed to compare the effectiveness of different PR exercise approaches in patients with F-ILDs.
View Article and Find Full Text PDFBMJ Open
May 2025
Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark.
Introduction: Fibrotic interstitial lung diseases (F-ILD) are severe and often progressive lung disorders that frequently lead to respiratory failure, with patients experiencing high symptom burdens, including severe dyspnoea. This is also evident in patients with severe chronic obstructive pulmonary disease (COPD). Many patients will eventually require ambulatory oxygen therapy (AOT) due to exertional desaturation.
View Article and Find Full Text PDF