COPD Research Articles

Loss of pulmonary capillaries in idiopathic pulmonary arterial hypertension with low diffusion capacity is accompanied by early diffuse emphysema detected by 129Xe MRI.

Recent studies suggest the existence of an idiopathic pulmonary arterial hypertension (IPAH) phenotype affecting mostly patients with a smoking history, characterised by low diffusion capacity for carbon monoxide (DLCO) without clinically significant emphysema. This study's objective was to test the hypothesis of a loss of pulmonary capillaries as an underlying mechanism by comparison to other patient groups with and without pulmonary hypertension (PH). Between March 2019 and June 2023, patients of four groups were recruited for this observational study: IPAH with preserved (1) and low DLCO (2), combined pulmonary fibrosis and emphysema with PH (3), and emphysema without PH (4). Patients underwent clinical CT and 129Xe MRI including dissolved-phase imaging yielding the ratio of 129Xe in red blood cells and membrane tissues (RBC-M), chemical shift saturation recovery for determining RBC fraction η and diffusion-weighted imaging yielding surface-volume ratio. Kruskal-Wallis tests were used for statistical analysis. Twenty-nine participants were recruited, of which 22 (age 64 ± 10, 11 male, 5/5/7/5 for the individual groups) could be included in the analysis. RBC-M and η were reduced in IPAH with low versus preserved DLCO and emphysema groups (p ≤ 0.01). CT low-attenuation area percentage was not increased in IPAH with low DLCO compared to any group. 129Xe MRI-derived surface-volume ratio was reduced in IPAH with low versus preserved DLCO (p = 0.04). Results are consistent with a loss of pulmonary capillaries in patients with IPAH and low DLCO along with destruction of alveolar tissue, likely due to early diffuse emphysema. Question A loss of pulmonary capillaries has been suggested in patients with IPAH and low diffusion capacity without clinically significant emphysema on CT. Findings 129Xe uptake in red blood cells and lung surface-volume ratio were reduced in IPAH patients with low compared to preserved diffusion capacity. Clinical relevance This study furthers the understanding of the underlying pathological mechanisms in IPAH with low diffusion capacity, providing evidence that loss of pulmonary capillaries is accompanied by alveolar tissue destruction despite near-normal CT.

Smoking-Related Interstitial Lung Disease: CT Image-Based Review and Update

Tobacco smoking is the leading cause of preventable deaths in the United States. Beyond the risks of cardiovascular disease and several cancers, smoking contributes to lung inflammation, lung destruction, and smoking-related interstitial lung diseases (SRILD). SRILD describes a broad range of conditions that includes respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, pulmonary Langerhans cell histiocytosis, acute eosinophilic pneumonia, idiopathic pulmonary fibrosis, combined pulmonary fibrosis and emphysema, interstitial lung abnormality, and smoking-related interstitial fibrosis. Computed tomography (CT) is central to the diagnosis and understanding of clinicopathologic manifestations of smoking-related lung injury. Common features of SRILD on CT include low-attenuation areas, ground-glass opacities, fibrosis, and lung nodules. Although the various SRILDs are often described as distinct entities, they may manifest with nonspecific features or exhibit mixed patterns and may more accurately be described as a continuum of pathology. Understanding the broad range of radiologic features and recognizing the potential coexistence and overlap of disease processes is essential to maintaining an appropriate differential diagnosis. In this article, we review the radiologic findings associated with SRILDs with a focus on diagnostic considerations and challenges when interpreting CT images.

An intelligent model of pulmonary emphysema detection using adaptive image segmentation and multi-dilated densenet with attention mechanism

Pulmonary emphysema is a significant factor in lung cancer and Chronic Obstructive Pulmonary Disease (COPD). Traditional pulmonary emphysema detection models may have difficulty in accurately detecting and diagnosing the severity of the disease. So, this work developed a novel pulmonary emphysema detection system with the help of deep learning frameworks. Originally, the significant images are accumulated from the benchmark sources, and fed into the Adaptive Trans-DenseUnet (ATDUnet)-based segmentation model. The ATDUnet model is highly effective in accurately segmenting pulmonary emphysema from the gathered images. Moreover, to enhance the segmentation process, the parameters are tuned in the ATDUnet using the Statistical Solution of the Osprey Optimization Algorithm (SSOOA). Subsequently, the segmented image is given to the pulmonary emphysema classification phase, where the Multi-Dilated DenseNet with Attention Mechanism (MDDNet-AM) is employed. By incorporating an attention mechanism, MDDNet-AM can focus on important features within the image for improved accuracy and efficiency in diagnosis. Finally, the developed model offered the pulmonary emphysema classified outcome. Then, the outcome of the developed model is compared against conventional pulmonary emphysema detection methods, and given the accuracy to be 93.10. Therefore, the result proved that the use of developed advanced technology in pulmonary emphysema detection has shown promising results in the field of respiratory health.

A novel chronic obstructive pulmonary disease mouse model induced by intubation-mediated intratracheal co-administration of porcine pancreatic elastase and lipopolysaccharide

BackgroundChronic obstructive pulmonary disease (COPD) is a significant respiratory disorder in humans characterized by persistent airway constriction or obstruction due to chronic bronchitis and pulmonary emphysema. Various methods of inducing COPD in mouse models are frequently used in COPD research; however, these cannot completely reproduce histopathologic lesions. This study aimed to establish a new COPD mouse model that reproduces histopathological lesions closely resembling clinical COPD within a shorter induction time.MethodsThe new strategy involved the co-administration of porcine pancreatic elastase (PPE) and lipopolysaccharide (LPS), with PPE intended to induce pulmonary emphysema and LPS intended to induce chronic bronchitis. Male C57BL/6J mice were administered PPE (8 U/kg) on days 0 and 3 and LPS (400 µg/kg) on days 6, 9, 12, and 15. Each administration was performed using a noninvasive intubation-mediated intratracheal instillation method with a laryngoscope.ResultsPostmortem examination on day 22 revealed that pulmonary emphysema and chronic bronchitis were simultaneously induced in 90.91% of the lung lobes. Molecular studies revealed higher messenger ribonucleic acid (mRNA) expression levels of interleukin-6(IL-6) and matrix metalloproteinase-12(MMP-12) associated with the pathogenesis of COPD.ConclusionA new method was developed to establish a COPD mouse model that displays a more severe representation of the histopathological findings of clinical COPD than previous COPD models. It also reduces the time required for model induction. This newly developed COPD mouse model is expected to be a valuable tool for the pathogenesis and therapeutic research on human COPD.

Clinical and biologic profiles of patients with acute respiratory distress syndrome by prevalence of chronic obstructive pulmonary disease or emphysema; a cohort study

IntroductionAcute respiratory distress syndrome (ARDS) is characterized by diffuse lung injury. The impact of pre-existing chronic obstructive pulmonary disease (COPD) or emphysema on ARDS pathogenesis is not well characterized.MethodsSecondary analysis of ARDS patients enrolled in the Acute Lung Injury Registry and Biospecimen Repository at the University of Pittsburgh between June 2012 and September 2021. Patients were categorized into two mutually exclusive groups by the prevalence of COPD or emphysema at the time of ARDS diagnosis. The COPD/emphysema group comprised ARDS patients with radiological evidence of emphysema, chart diagnosis of COPD, or both. Demographics, lung mechanics, and clinical outcomes were obtained from the electronic medical record. Host-response biomarkers known to have validated associations with ARDS were previously measured in plasma and lower respiratory tract samples using a customized Luminex assay. Continuous and categorical variables were compared between groups with and without COPD/emphysema.Results217 patients with ARDS were included in the study, 57 (27%) had COPD/emphysema. Patients with COPD/emphysema were older (median 62 [interquartile range 55–69] versus 53 [41–64] years, p < 0.01), more likely to be male (62% vs. 44%, p = 0.02) and had a higher prevalence of congestive heart failure (25% vs. 4%, p < 0.01) compared to patients without COPD/emphysema. Baseline demographics, laboratory parameters, and mechanical ventilatory characteristics were otherwise similar between the two groups. No difference in 90-day mortality was observed between groups; however, patients with COPD/emphysema had shorter duration of intensive care unit (ICU) stay (median 10 [7–18] versus 16 [9–28] days, p = 0.04) and shorter duration of mechanical ventilation (median 7 [4–16] vs. 12 [6–20] days, p = 0.01). Host response biomarkers in serum and lower respiratory tract samples did not significantly differ between groups.ConclusionARDS patients with COPD or emphysema had similar respiratory mechanics, host response biomarker profiles, and mortality compared to those without COPD or emphysema but with a shorter median duration of mechanical ventilation and ICU length of stay. Future studies should address differences in clinical and biological responses by disease severity, and should investigate the impact of severity of COPD and emphysema on mechanical ventilation and targeted therapeutic strategies in ARDS.Clinical trial numberNot applicable

“Challenging acquired pulmonary interstitial emphysema in an extreme preterm infant: A case report and review of literature”

Background Pulmonary interstitial emphysema (PIE) can occur in the immature lungs of preterm infants due to overdistension from mechanical ventilation. Case Report Preterm female infant (26 weeks of gestational age, 950 grams) born via urgent cesarean section, APGAR 1/3/6. Invasive mechanical ventilation during the first day of life transitioned to noninvasive positive pressure ventilation (NIPPV), afterward initially stable. Apnea and bradycardia noted at 20 days of life, requiring endotracheal intubation, with complementary studies showing right PIE. Progressive emphysema deterioration, air trapping, and left lung atelectasis, with hemodynamic repercussion despite positioning, high-frequency oscillatory ventilation, and hemodynamic support with dopamine and dobutamine. Selective left intubation attempted positioning the infant, unsuccessful. Due to failure of conventional therapies and impossibility to achieve selective intubation, pediatric surgery performed endobronchial balloon occlusion with angioplasty balloon inflated with contrast with fluoroscopy control. Progressive improvement, weaning ventilatory settings with acceptable oxygenation. Balloon was retired after 5 days. Mechanical ventilation was discontinued at 52 days of life. Infant was discharged without respiratory support nor oxygen at 100 days of life. Conclusions PIE treatment is based in conventional measures (respiratory support and positioning) but eventually might require alternative techniques when the first fail, with limited work in pediatric population and further technical limitations in the preterm infants.

Lung transplantation for alpha-1-antitrypsin deficiency and Behçet's disease: A case report

INTRODUCTIONAlpha-1-Antitrypsin deficiency (A1ATD) is a genetically determined anti-proteinase deficiency which predisposes to early onset emphysema and liver disease. Lung transplantation (LTx) is the final therapeutic option. Behçet's disease (BD) is a rare autoimmune disease characterized by oral and genital ulcers, deep venous thrombosis associated with large and small-vessel vasculitis and aneurysms. The association of A1ATD and BD is unknown. We aim to describe a rare case of concomitant presentation of these pathologies in a patient submitted to LTx. CASE REPORTA 31-years-old female presented with oral and genital ulcers associated with cavernous sinus thrombosis in 2012, being diagnosed with BD. In 2018 debuted with progressive respiratory symptoms. CT-scan revealed extensive pulmonary emphysema associated with decreased pulmonary function. A1ATD was identified with a heterozygous “MZ” allelic combination. She was referred to LTx center in 2021. After additional investigation, the patient was considered to LTx. She underwent standard bilateral LTx in July of 2022. During post-operative time, special attention was given to the risk of vascular complications, and pos-operative angio-CT was performed actively searching for this possible outcome. Nevertheless, no major events occurred. After 1 year, she is fully functional with no signs of BD activity. CONCLUSIONTo our knowledge, this is the first description of LTx for A1ATD in a patient with BD, proving its feasibility in a highly selected patient at an experienced and specialized center. Nonetheless, solid organ transplantation in patients with BD remains a high risk procedure and should be indicated with caution.

Associations of pulmonary microvascular blood volume with per cent emphysema and CT emphysema subtypes in the community: the MESA Lung study

BackgroundPulmonary microvasculature alterations are implicated in emphysema pathogenesis, but the association between pulmonary microvascular blood volume (PMBV) and emphysema has not been directly assessed at scale, and prior studies have...

The Potential Role of Cigarette Smoke, Elastic Fibers, and Secondary Lung Injury in the Transition of Pulmonary Emphysema to Combined Pulmonary Fibrosis and Emphysema.

Combined pulmonary fibrosis and emphysema (CPFE) is a distinct syndrome associated with heavy smoking. The fibrotic component of the disease is generally believed to be superimposed on previously existing pulmonary emphysema, but the mechanisms responsible for these changes remain poorly understood. To better understand the pathogenesis of CPFE, we performed a series of experiments that focused on the relationships between lung elastic fibers, cigarette smoke, and secondary lung injury. The results indicate that even brief smoke exposure predisposes the lung to additional forms of lung injury that may cause alveolar wall fibrosis. The proinflammatory activity of smoke-induced structural alterations in elastic fibers may contribute to this process by enhancing secondary lung inflammation, including acute exacerbations of chronic obstructive pulmonary disease. Furthermore, the levels of the unique elastin crosslinks, desmosine and isodesmosine, in blood, urine, and sputum may serve as biomarkers for the transition from pulmonary emphysema to interstitial fibrosis. While the long-term effects of these inflammatory reactions were not examined, the current studies provide insight into the potential relationships between elastic fiber injury, cigarette smoke, and secondary lung injury. Determining the mechanisms involved in combined pulmonary emphysema and fibrosis and developing a sensitive biomarker for this type of lung injury may permit timely therapeutic intervention that could mitigate the high risk of respiratory failure associated with this condition.

An underrecognized phenotype of pulmonary emphysema with marked pulmonary gas exchange but with mild or moderate airway obstruction

In patients with pulmonary emphysema and mild to moderate airflow limitation, one does not expect the features marked exertional dyspnea and hypoxemia as well as a profound decrease in diffusing capacity of the lung for carbon monoxide (DLCO). Here we describe this phenotype and its prognosis. From our database, we retrospectively selected cases associating emphysema, exertional breathlessness, O2 requirement at least upon exercise, forced expiratory volume in 1 sec (FEV1) ≥ 50% predicted, and DLCO ≤ 50% predicted, without associated combined pulmonary fibrosis and emphysema, right-to-left shunt, or severe pulmonary hypertension. Over a 12-year period, we identified 16 patients with emphysema and the above presentation. At the initial evaluation, the median age was 62 years (interquartile range 53.8–68.9). The median FEV1 and DLCO% predicted and mean pulmonary artery pressure were 86 (65–95)%, 38 (31–41)%, and 20 (17–25) mm Hg, respectively. On room air, the median arterial partial pressure of oxygen and partial pressure of carbon dioxide in arterial blood were 63.5 (55.8–69) mm Hg and 34.5 (31–36) mm Hg with increased median alveolar-arterial oxygen difference (46 [39–51] mm Hg). After the initial evaluation, the respiratory condition worsened in 13 of 14 (92.8%) patients with one or more re-evaluations (median follow-up 2.6 [0.9–5.8] years). In 12, lung transplantation was considered. Four patients died after 5.8, 5.7, 7.1, and 0.8 years of follow-up, respectively. We describe an underrecognized phenotype of pulmonary emphysema featuring a particular profile characterized by marked exertional dyspnea, impaired pulmonary gas exchange with low DLCO and marked oxygen desaturation at least on exercise but with mild or moderate airway obstruction.

The Influence of Emphysema on Treatment Response to Biologic Therapy in Severe Asthma.

Patients with severe asthma (SA) benefit from biologic therapy substantially. However, the impact of smoking-related comorbidities remains unclear due to the exclusion of patients with ≥10 pack-years from asthma studies. Our aim was to examine the effects of emphysema on biologic treatment response in SA in this retrospective cohort study. Pulmonary emphysema was examined using computed tomography. Patients with SA were included and divided into two groups based on emphysema quantity (≥5% or <5%). They received either anti-IgE (22.1%), anti-IL-5-(receptor) (52.3%), or anti-IL-4/IL-13 (25.6%) biologic therapy. Treatment response was assessed after 7.8 ± 2.5 months based on acute exacerbations (AE), oral corticosteroid (OCS) therapy, Asthma Control Test (ACT), forced expiratory volume in 1 second (FEV1) and using the Biologics Asthma Response Score (BARS). This study comprised 86 patients (mean age 56.1 ± 12.8 years; 54% female). Half (43, 50.0%) were never-smokers, half ex-smokers with an average of 26.9 ± 18.2 pack-years. Patients with ≥5% emphysema were more often ex-smokers (80% vs 41%, p=0.002), had poorer lung function (FEV1 median 1.3 [interquartile range: 1.0;1.6] vs 1.8[1-2;2.4] L, p=0.037), and more comorbid COPD (50% vs 21%, p=0.012). However, no significant differences were noted in treatment response regarding annualized AE rate (-2.5[-5;-1] vs -3.0[-5;-2] n/year, p=0.295) and OCS reduction (-4[-10;0] vs -5[-10;0] mg, p=0.691), ACT score (5[3;9] vs 4[0;9] points, p=0.579) or FEV1 improvement (0.03[-0.15;0.25] vs 0.23[-0.5;0.49] L, p=0.052), BARS (p=0.312), and remission rates (15.0% vs 19.7%, p=0.753). In patients with severe asthma, those with comorbid emphysema show similar treatment response to biologic therapy. Therefore, suitable patients should not be denied biologics due to the presence of emphysema.

Combined pulmonary fibrosis and emphysema and lung transplantation: current evidence and future directions

Combined pulmonary fibrosis and emphysema and lung transplantation: current evidence and future directions

Assessment of Oral Bacterial Microflora in Patients with Stable Chronic Obstructive Pulmonary Disorders

Background &amp; Objectives: Chronic obstructive pulmonary disorder is an inflammatory disease characterized by the progressive deterioration of pulmonary function and increasing airway obstruction, includes chronic bronchitis and emphysema. The oral cavity has been considered a potential reservoir for respiratory pathogens. Oral bacteria may play a vital role in the exacerbation of chronic obstructive pulmonary diseases. Hence, we intend to study the bacterial microflora of oral cavity in stable COPD patients. Methods: In this study 20 patients aged between 20 to 60 years diagnosed with stable COPD and 20 normal healthy controls were selected based on the set inclusion and exclusion criteria. A detailed case history, physical examination and investigations and all the subjects were examined for gingival and periodontal status by recording the Oral hygiene which comprises debris index and calculus index and periodontal index and Quantitative oropharyngeal cultures were obtained by oral washing using isotonic saline as the collecting fluid. Results: A significant higher score of OHI(S) and PI was observed in COPD patients. There is a direct correlation between periodontal pathogens with the severity of COPD. Conclusion: The predominant organisms found in the oropharyngeal secretions were porphyromonas gingivalis and haemophilus influenza, in COPD patients.

Incidence and factors associated with mitral valve reoperation in patients undergoing intervention for mitral regurgitation: a nationwide cohort study

Abstract Background When intervention is needed for the treatment of mitral regurgitation, the risk of reoperation is of concern for both patients and physicians. As such, contemporary and nationwide data on the incidence and factors associated with mitral reoperation can provide crucial information to support patient education and clinical follow-up after intervention. Purpose To examine the incidence of mitral reoperation in patients operated for mitral regurgitation. Second, to assess whether reoperation rates differed according to time since surgery and identify potential factors associated with reoperation. Methods Using Danish nationwide registries, patients undergoing first-time surgery for mitral regurgitation 1996–2021 were identified. Exclusion included first-time surgery due to endocarditis. According to index surgery, the population was categorized into 1) mitral repair; 2) mechanical prosthesis; 3) bioprosthesis. Patients were followed from date of discharge. Cumulative incidence of reoperation was examined using competing risk analysis during a maximum of 20-years follow-up. Crude incidence rates of reoperation were reported from 0-6 months and for overall study period. Multivariate Cox analysis was used to examine factors associated with reoperation. Results We identified 7,063 patients: 4,680 (66.3%) with repair (72% male, median age 66), 1,273 (18.0%) with mechanical prosthesis (52% male, median age 59), and 1,110 (15.7%) with bioprosthesis (57% male, median age 74). Median follow-up was 7.2 years, 10.7 years, and 4.5 years, respectively. At end of follow-up, cumulative incidences were 7.7% for repair, 6.8% for mechanical prostheses, and 7.5% for bioprostheses. Of these, reoperation was preceded by endocarditis in 8.0%, 21.5%, and 30.4% of the respective patient groups. The cumulative incidence function demonstrates that the highest proportion of reoperations occurred within first six months, Figure 1. From 0-6 months following surgery, incidence rates per 100 person-years were 9.1 for repair, 11.7 for mechanical prostheses, and 18.4 for bioprostheses, while this was 8.6, 9.4, and 17.0, respectively for the overall study period. Factors associated with increased rate of reoperation were heart failure (HR=1.80, 95% CI 1.40-2.32), chronic renal failure (HR=1.48, 95% CI 1.11-1.98) and chronic obstructive pulmonary disorder (COPD) (HR=1.34, 95% CI 1.01-1.77), Figure 2. Compared to mitral repair, mechanical prostheses were associated with lower rate of reoperation (HR=0.57, 95% CI 0.42-0.78), while there was no statistically significant difference between repair and bioprostheses (HR=1.10, 95% CI 0.81-1.50). Conclusion In patients operated for mitral regurgitation, cumulative incidences of reoperation with a maximum of 20-year follow-up were 7.7% for repair, 6.8% for mechanical prostheses, and 7.5% for bioprostheses. Comorbidities such as heart failure, renal failure and COPD were associated with increased risk of reoperation.Figure 1Figure 2

Rheumatoid arthritispresenting by combined pulmonary fibrosis and emphysema with clubbing and pulmonary hypertension: A rare case report.

A 60-year-old male smoker came with dyspnea. A rare condition known as combined pulmonary fibrosis and emphysema (CPFE) was observed on a chest CT scan. Decreased DLCO in combination with normal FVC can help diagnose CPFE. Concomitant connective tissue diseases, including rheumatoid arthritis, should be checked in CPFE patients.

The impact of pulmonary fibrosis on cardiac function in chronic obstructive pulmonary disease

Abstract Background Emerging evidence indicates the coexistence pulmonary fibrosis and emphysema in patients with Chronic Obstructive Pulmonary Disease (COPD). The effect of pulmonary fibrosis on cardiac structure and function in COPD patients remains unclear. Purpose This study aimed to examine the association between the degree of pulmonary fibrosis identified on Computed Tomographic (CT) imaging and echocardiographic measures of cardiac structure and function in individuals with COPD. Methods The study included 456 individuals with COPD, participating in a large prospective cohort study. Patients with available CT scans and echocardiograms were included. Local histogram-based assessments of lung density were utilized to determine the percentage of fibrosis. Fibrosis was defined based on the CT attenuation within the range of -600 and -250 Hounsfield units. The association between percent fibrosis and measures of cardiac structure and function were investigated by univariable and multivariable linear regression models. Multivariable adjustments included age, sex, body mass index (BMI), and smoking. In addition, restricted cubic splines were generated to visually present the relationship between the percentage of fibrosis and specific echocardiographic parameters. Results The mean age was 71 (± 8 years), 48% were female, and the median BMI was 27 kg/m2 (IQR: 22; 30kg/m2). The mean forced expiratory volume in 1 second as a percentage of the predicted value (FEV1pp) was 47% (±18%). The mean forced vital capacity of predicted value was 71% (± 19%). The median percentage of fibrosis in the cohort was 4.9% (IQR: 3.9; 6.0%). Patients were stratified into tertiles based upon fibrosis percentage. BMI, frequency of diabetes, and FEV1pp were increasingly positively associated with fibrosis. Furthermore, significant trends for echocardiographic measures found that the patients with the most extensive fibrosis were seen to have impaired left ventricular (LV) ejection fraction (LVEF), lower absolute global longitudinal strain (GLS), increased left atrial (LA) volume index (LAVi), and higher LV mass as compared to those with less significant fibrosis. There were no significant differences between tertiles of fibrosis and age or sex. In univariable linear regression, significant inverse associations between the percentage of fibrosis, LVEF, GLS, and tricuspid annular plane systolic excursion (TAPSE) were seen (Table). For LV mass index and LAVi, significant direct associations were found (Table). After adjusting for potential confounding factors, only GLS and LAVi remained associated with pulmonary fibrosis (Figure). Conclusion This study found that GLS and LA volume were significantly associated with the degree of pulmonary fibrosis, even after adjusting for confounding variables. This may suggest that fibrosis is primarily linked with impaired left-sided measures of cardiac structure and function.Linear Regression ModelsCubic Spline Curves

Acute vasoreactivity testing for severe pulmonary hypertension associated with lung disease

Abstract Background Pulmonary hypertension results in clinical deterioration and increases mortality risk in patients with lung disease (LD), including chronic obstructive pulmonary disease, interstitial lung disease and combined pulmonary fibrosis and emphysema. Though acute vasoreactivity testing predicts long term-survival of patients with pulmonary arterial hypertension (PAH), its clinical impact for patients with pulmonary hypertension associated with LD (LD-PH) remains unknown. Purpose The purpose of this study is to clarify the clinical impact of acute vasoreactivity testing using inhaled nitric oxide for severe LD-PH. Methods We retrospectively reviewed 65 consecutive patients with LD-PH between January 2014 and December 2022; 48 patients (73 [68–78] years; males, 81%; median follow-up period 23 [7–35] months) with severe pulmonary hypertension who underwent acute vasoreactivity testing were included. We divided the patients based on a median pulmonary vascular resistance (PVR) response (−15%) in acute vasoreactivity testing into the high-responder (PVR response ≦−15%) and the low-responder (PVR response &amp;gt;−15%) groups. Results Acute vasoreactivity testing significantly decreased mean pulmonary arterial pressure (mPAP) (from 41 [34–48] to 36 [31–40] mmHg, P&amp;lt;0.01) and PVR (from 10.7 [8.3–13.8] to 8.9 [6.6–11.4] Wood units, P&amp;lt;0.01). The high-responder group had better long-term survival than the low-responder group (P = 0.03). Univariate Cox regression analysis showed that the prognostic factors for better survival included younger age, high-responder to acute vasoreactivity testing, and PAH-specific therapy (P &amp;lt;0.01, 0.04, 0.03, respectively). There were correlations between the PVR response at acute vasoreactivity testing and improvement in mPAP (R=0.40, P=0.03) or PVR (R = 0.47, P &amp;lt;0.01) with PAH-specific therapy. Conclusions PVR response at acute vasoreactivity testing was associated with better long-term survival and haemodynamic improvement with PAH-specific therapy. Acute vasoreactivity testing may be useful to predict the responders to PAH-specific therapy and long-term survival in patients with severe LD-PH.

Intermittent Exercise and Cigarette Smoke Extract-Induced Pulmonary Tensegrity Dysfunction; Monitoring Elastin, Matrix Metalloproteinase-12, Transforming Growth Factor-beta 1

Background: Cigarette smoke causes inflammation of the alveoli, leading to airflow restriction and potentially irreversible changes in lung architecture, including pulmonary emphysema. This study investigates the effects of a six-week aerobic interval training regimen on elastin activity in a rat model of cigarette smoke-induced COPD (CS-COPD). Objectives: Specifically, we observe the variables matrix metalloproteinase-12 (MMP12) and transforming growth factor-beta (TGF-β1). Methods: Twenty male Wistar rats, aged eight weeks, were divided into four groups (n = 5 per group): Control, aerobic interval training (Exe), cigarette smoke extract (CSE), and CSE + Exe. The aerobic interval training (Exe) was performed for 49 min/day, five days per week for six weeks in interval form. The CSE group received an intraperitoneal (IP) injection of 150 µL of cigarette smoke extract, one day per week for six weeks. Results: The CSE and CSE + Exe groups showed a significant decrease in elastin gene expression in lung tissue compared to the healthy control group (P = 0.0002 and P = 0.0057, respectively) and the Exe group (P = 0.0002 and P = 0.0042, respectively). Additionally, the CSE group exhibited a significant increase in lung MMP12 gene expression compared to the healthy control and Exe groups (P = 0.0008 and P = 0.0013, respectively). Notably, the CSE + Exe group showed a significant decrease in MMP12 gene expression in lung tissue compared to the CSE group (P = 0.0015). Furthermore, the CSE and CSE + Exe groups demonstrated a significant increase in TGFB1 gene expression in lung tissue compared to the healthy control group (P &lt; 0.0001 and P = 0.0022, respectively) and the Exe group (P &lt; 0.0001 for both). Conclusions: Our findings indicate that the group exposed to cigarette smoke experienced a significant decrease in elastin levels, accompanied by an increase in MMP12 and TGF-β1 expression. Although aerobic interval training provided some protection against the detrimental effects of cigarette smoke exposure, it was not sufficient to fully reverse the harmful impacts of the disease.

Pulmonary Emphysema Disease Classification Model Using Deep Structured Techniques

Emphysema is normally employed in the sense of morphology, and thus, imaging the modalities has a significant part in detecting the disorder. Most importantly, the High-Resolution Computed Tomography (HRCT) is a trustworthy component for illustrating the emphysema pathology. Hence, the pulmonary emphysema diagnosis at the starting stage is very important and can secure the life of a human. When there is a failure in the technique, there is a possibility of the death that displays the importance of effective diagnosis. The endotracheal intubation’s delay is prevented by implementing distinct mechanisms that result in various requirements. The presented work develops a deep learning-aided pulmonary emphysema disease classification model that is implemented to defeat the above difficulties. At first, the images are accumulated from a standard dataset. Then, the collected images are provided to the phase of segmentation. Here, the newly developed Adaptive Trans-Residual Dense UNet (ATRDUNet) is employed to perform the segmentation process. Also, the recommended ATRDUNet model helps to locate the disease in the appropriate location. Moreover, the performance of the segmentation process is enriched to fine-tune the parameter using the Fitness Revised Position of Wild Horse Optimizer (FRP-WHO). While optimizing the parameters effectively, it tends to handle the complex issues to strengthen the model accurately. The segmented results from the ATRDUNet are passed to Vision Transformer-based Residual DenseNet with LSTM layer (ViTrans-RDLSTM) for getting the final classified outcome. To this end, the analysis of the suggested model is correlated to find the effectiveness of the recommended model. However, these results are examined with the help of standard performance measures. Also, the experimentation is carried out using the standard “Automatic emphysema detection using weakly labeled HRCT lung images” dataset to attain better outcomes in the developed model. The findings of the recommended approach show 95% in terms of accuracy. Also, the statistical analysis of the developed model shows 23.9%, 12.3%, 15.2%, and 13.0% better performance than BWO, CO, HBA, and WHO in terms of mean analysis to show enriched performance.

Correction to: Pulmonary Interstitial Emphysema in an Infant with Critical Congenital Heart Defect Associated with TNNC1 Gene Mutation and 22q11.2 Microdeletion: a Case Report

Correction to: Pulmonary Interstitial Emphysema in an Infant with Critical Congenital Heart Defect Associated with TNNC1 Gene Mutation and 22q11.2 Microdeletion: a Case Report