Fibrotic lung diseases are often characterized by chronic inflammation and the progressive destruction of the vasculature, parenchyma, and airways, leading to cellular metabolic changes. As a result, these changes activa...Fibrotic lung diseases are often characterized by chronic inflammation and the progressive destruction of the vasculature, parenchyma, and airways, leading to cellular metabolic changes. As a result, these changes activate several pathological pathways, contributing to the disease's progression and worsening. However, the precise impact of metabolic changes and their contributions to the progression of fibrotic lung diseases need deeper exploration. The current review highlights the interplay between immunometabolites and hypoxia in bringing out cellular and epigenetic changes that progress and further exacerbate pulmonary fibrosis. Notably, the mitochondrial-linked immunometabolites such as lactate, succinate, 2-hydroxyglutarate (2-HG), fumarate, and itaconate have the potential to determine cellular fate in health and disease. For instance, lactate accumulation is one of the vital factors associated with pulmonary fibrosis (PF). The metabolite succinate promotes hypoxia response, inflammatory markers accumulation, fibroblast activation, and PF, whereas L-2-HG impairs the TCA cycle, reduces glycolysis, and disrupts the nicotinamide adenine dinucleotide (NADH/NAD+) ratio, ultimately leading to dysfunctional mitochondrial respiration and contributing to lung fibrosis. Due to the progressive and degenerative nature of fibrotic lung diseases, individuals affected by them need ongoing clinical support and monitoring. The currently available pharmacological treatments are limited and come with multiple side effects. Therefore, the search for newer therapeutics in the form of small molecules targeting these metabolites is increasingly being formulated to treat chronic fibrotic pulmonary conditions through their exhaustive mechanistic investigations backed by robust preclinical and clinical trials.
This systematic review and meta-analysis evaluated the performance of machine learning (ML) models in predicting mortality among pulmonary embolism (PE) patients, synthesizing data from 17 studies encompassing 844,071 ca...This systematic review and meta-analysis evaluated the performance of machine learning (ML) models in predicting mortality among pulmonary embolism (PE) patients, synthesizing data from 17 studies encompassing 844,071 cases. Logistic Regression was the most commonly used algorithm, followed by advanced models like Random Forests, Support Vector Machines, XGBoost, and Neural Networks. Pooled performance metrics from 12 studies demonstrated a sensitivity of 0.88 (95% CI: 0.78-0.94, = 90.43%), specificity of 0.79 (95% CI: 0.62-0.89, = 99.53%), positive likelihood ratio of 4.1 (95% CI: 2.2-7.7), negative likelihood ratio of 0.16 (95% CI: 0.08-0.29), diagnostic odds ratio of 26 (95% CI: 10-71), and an AUROC of 0.91 (95% CI: 0.88-0.93), indicating excellent discriminative ability. Subgroup analyses revealed higher sensitivity in advanced ML models (89.7%) and non-USA studies (97.2%), with advanced ML showing lower specificity heterogeneity ( = 0%). Significant heterogeneity was observed, particularly in specificity ( = 99%), driven by traditional ML and USA-based studies. Minimal publication bias was noted for sensitivity (Egger's = 0.942), but specificity showed potential bias (Egger's = 0.038 after outlier exclusion). These findings suggest that ML models outperform traditional risk stratification tools in predicting PE mortality, offering robust potential for clinical decision-making, though heterogeneity and retrospective study designs warrant cautious interpretation. PROSPERO: CRD420251026696.
Pulmonary hypertension (PH) is a severe complication observed in pediatric patients after hematopoietic cell transplantation or chemotherapy. A review of records at Hokkaido University Hospital (2014-2024) identified fou...Pulmonary hypertension (PH) is a severe complication observed in pediatric patients after hematopoietic cell transplantation or chemotherapy. A review of records at Hokkaido University Hospital (2014-2024) identified four cases of PH, each with different etiologies, including pulmonary arterial hypertension, pulmonary veno-occlusive disease, and microthromboembolism. Contributing factors included splenic atrophy, corticosteroid-responsive inflammation, and potential drug-induced vascular remodeling. Although transient PH usually resolves, late-onset PH emphasizes the need for long-term echocardiographic monitoring. These findings underscore the importance of individualized management, avoiding pulmonary vasodilators without proper evaluation, and addressing underlying conditions such as thrombotic microangiopathy or interstitial lung disease.
Pulmonary hypertension (PHTN) in infants with developmental lung disease, such as bronchopulmonary dysplasia (BPD), chronic lung disease of infancy (CLD), or congenital diaphragmatic hernia (CDH), can be exacerbated by a...Pulmonary hypertension (PHTN) in infants with developmental lung disease, such as bronchopulmonary dysplasia (BPD), chronic lung disease of infancy (CLD), or congenital diaphragmatic hernia (CDH), can be exacerbated by atrial septal shunts secondary to atrial septal defects (ASD). While transcatheter ASD closure may reduce pulmonary overcirculation, data on post-closure hemodynamic and pharmacologic outcomes remain limited. This single-center retrospective study aimed to characterize changes in PHTN severity, respiratory support, and medication use 1 year after early transcatheter ASD closure (defined as closure at ≤ 1 year of age). Eligible patients were infants with BPD, CLD, or CDH who underwent early transcatheter ASD closure between 2021 and 2024 and had preprocedural PHTN medication use and respiratory support. Sixteen infants met the inclusion criteria. At 1 year, excluding the 3 who died, 10 of 13 infants (76.9%) showed improved PHTN severity, including 6 (60%) with complete resolution. Of the 13 infants, 6 (46.2%) weaned off all respiratory support. Average diuretic dosage (mg/kg/day) decreased by 92.9%, and vasodilator dosage declined by 47.0%. Infants with ASDs ≥ 5 mm and gestational age (GA) < 32 weeks required significantly longer diuretic therapy than those with smaller ASDs (< 5 mm) and GA ≥ 32 weeks. No similar associations were found with vasodilator weaning. These findings suggest early transcatheter ASD closure may offer therapeutic benefit in select high-risk infants, resulting in improved hemodynamics and reduced medication dependence. Although limited by small sample size and retrospective design, this study supports the potential for individualized weaning strategies and the need for prospective multicenter investigations.
Adhesive reactions are common complications of continuous treprostinil infusions, limiting their recommended use as a treatment for severe pulmonary hypertension. We present the case of a 10-year-old male with recurrent...Adhesive reactions are common complications of continuous treprostinil infusions, limiting their recommended use as a treatment for severe pulmonary hypertension. We present the case of a 10-year-old male with recurrent adhesive-related skin reactions, which compromised both subcutaneous and intravenous continuous treatment. Due to comorbid atopic dermatitis (AD), dupilumab was initiated to decrease skin reactions to adhesives. Within 48 h of initiation, skin reactions completely resolved. With sustained dupilumab treatment, he remains symptom-free and is successfully tolerating intravenous treprostinil infusion.
The activin signaling inhibitor sotatercept was approved for Group 1 pulmonary arterial hypertension (PAH) based on Phase 2 and 3 clinical trials showing significant improvements in primary outcomes; reduced pulmonary va...The activin signaling inhibitor sotatercept was approved for Group 1 pulmonary arterial hypertension (PAH) based on Phase 2 and 3 clinical trials showing significant improvements in primary outcomes; reduced pulmonary vascular resistance (PVR) and increased 6-min walk distance (6MWD), respectively. However, the efficacy and safety of transitioning off background therapies, including infusion prostacyclins, in patients receiving sotatercept are currently unknown. We report here a patient who was enrolled in sotatercept clinical trials (STELLAR/SOTERIA); during this period, he gradually transitioned from intravenous treprostinil. Subjective, physiologic, echocardiographic, and hemodynamic data after 2.5 years without intravenous therapy are presented. These results suggest that weaning off intravenous PGI2 may be feasible in some patients, but questions remain about the durability of the response and possible long-term adverse side effects.
Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction, proliferation, fibrosis, and microthrombosis of the pulmonary vasculature, which causes elevated pulmonary arterial pressure and vascular resist...Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction, proliferation, fibrosis, and microthrombosis of the pulmonary vasculature, which causes elevated pulmonary arterial pressure and vascular resistance leading to right ventricular failure and death. Previous treatments targeted three known pathways involved in the development of PAH: endothelin, nitric oxide, and prostacyclin. Dysfunctional signaling of the transforming growth factor-beta (TGF-β) family, via bone morphogenetic protein (BMP) receptor 2 and activin signaling, has also been implicated in PAH leading to the development of a new class of therapies. Sotatercept, the first medication in this class, has shown significant promise as an add-on therapy. Another drug in the class, cibotercept, is under investigation; however, the clinical trial has been suspended because of the development of pericardial effusions in some patients. In light of this new finding, we investigated whether our patients treated with sotatercept also experienced this possible side effect.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but serious disease caused by persistent thromboembolic obstruction or narrowing of the pulmonary arteries. Its prevalence varies, and many cases remain und...Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but serious disease caused by persistent thromboembolic obstruction or narrowing of the pulmonary arteries. Its prevalence varies, and many cases remain undiagnosed, contributing to a substantial clinical burden. This study aimed to summarize all CTEPH cases diagnosed in Latvia in 2024, calculate the annual incidence, and present additional epidemiological data from Latvian pulmonary hypertension (PH) registry. Additionally, we sought to document the presence of risk factors in individual patients and analyze their diagnostic pathways leading to a diagnosis of CTEPH. This observational study aimed to analyze diagnostic pathways and identify risk factors in all patients diagnosed with CTEPH in Latvia in 2024. Diagnosis was confirmed at Pauls Stradins Clinical University Hospital according to ESC guideline criteria. Patients completed the emPHasis-10 questionnaire and underwent a structured interview. In 2024, 15 patients were diagnosed with CTEPH in Latvia, corresponding to an annual incidence of 8.01 cases per million. Prevalence was 31.51 per million, and mortality-3.74 per 100 person-years. Patients diagnosed within 2 years of symptom onset had lower pulmonary vascular resistance compared to those diagnosed later. The most common risk factor was prior acute pulmonary embolism (80%). In 60% of cases, diagnosis was delayed by more than 2 years, reflecting late referral to a PH centre. CTEPH remains frequently misdiagnosed and undertreated. Improved recognition of risk factors and stronger collaboration with specialized PH centres are essential to optimize patient management and outcomes.
Pulmonary hypertension is common in children with Trisomy 21, frequently with multifactorial aetiologies. Registry data provide better understanding of disease development, diagnostic workup and treatment patterns in chi...Pulmonary hypertension is common in children with Trisomy 21, frequently with multifactorial aetiologies. Registry data provide better understanding of disease development, diagnostic workup and treatment patterns in children with Trisomy 21. TOPP (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) is a centre-based, comprehensive registry. Patients aged between 3 months and 18 years at time of diagnosis were eligible if they met predefined haemodynamic criteria. Demographic data, clinical symptoms at presentation, diagnostic tools, etiology, hemodynamic data, treatment, and follow-up were collected from the data base. Differences between the Trisomy 21 group and the non-Trisomy 21 group were analysed by the non-parametric Mann-Whitney test. Categorical variables were compared using the chi-squared test, or Fisher's exact test in the case of low expected frequencies. Out of 531 children in the registry, 62 patients (11.7%) were diagnosed with Trisomy 21. Compared to children without Trisomy 21, those with Trisomy 21 were younger at diagnosis, and had more often an associated congenital heart disease. Clinical symptoms at diagnosis were similar in children with or without Trisomy 21. However, those with Trisomy 21 presented less frequently with dyspnea with exertion, but more frequently with cyanosis, either at rest or with exertion. A comprehensive diagnostic workup in all children with Trisomy 21 was not done. Children with Trisomy 21 had lower mean pulmonary artery pressure (median 50 mmHg, IQR 38-62) and similar indexed pulmonary vascular resistance (median 11.5 WU.m, IQR 7.4-18.4) compared to patients without Trisomy 21. Children with Trisomy 21 were treated less frequently with targeted therapies for pulmonary arterial hypertension and received less combination therapy. In children with Trisomy 21 and pulmonary hypertension, early systematic diagnostic work up is essential to obtain the correct underlying pathology and guides to appropriate treatment.
Pediatric pulmonary arterial hypertension (PAH) has a long asymptomatic period with progressive vascular loss. A recent computational model of simulated PAH in humans has demonstrated that up to 70% of the pulmonary vasc...Pediatric pulmonary arterial hypertension (PAH) has a long asymptomatic period with progressive vascular loss. A recent computational model of simulated PAH in humans has demonstrated that up to 70% of the pulmonary vasculature is lost before clinical PAH criteria are met. We used this model in pediatric subjects with PAH to evaluate whether estimated pulmonary vascular loss or compromise (PVC) was associated with hemodynamic variables, survival, and other clinical outcomes. Retrospective and prospective cohort data were collected for subjects with PAH between 1999 and 2022 treated at our center. Cardiac catheterization and clinical data were compared with PVC estimated by the computational model. Transplant-free survival was associated with lower PVC (72% vs. 88%, < 0.001) and was also associated with a decrease in PVC over time with no significant change in PVC in subjects who died or underwent transplant. By Kaplan-Meier analysis, 10-year survival was 54% (IQR 35%, 81%) when PVC was more than 80%, compared with 100% survival (IQR 100%, 100%) when PVC was less than 80% ( < 0.001). By Cox proportional hazard regression, PVC was associated with mortality (HR 1.44, = 0.008). Lower PVC was associated with better clinical outcomes including percent predicted 6-min walk distance, brain natriuretic peptide, and estimated 1-year mortality. These findings demonstrate that PVC is a new computational hemodynamic variable estimating vascular area loss and is associated with transplant-free survival and other clinical outcomes in pediatric PAH. Further, PVC provides an adjunctive tool to potentially capture pulmonary vascular loss early in disease, progression, and response to therapy.
Pulmonary emboli (PE) are a common clinical problem seen when a peripheral deep vein thrombosis (DVT) migrates to the pulmonary arteries. However, emerging literature suggests that not all filling defects in the pulmonar...Pulmonary emboli (PE) are a common clinical problem seen when a peripheral deep vein thrombosis (DVT) migrates to the pulmonary arteries. However, emerging literature suggests that not all filling defects in the pulmonary arteries are the result of embolism, and that in situ pulmonary arterial thrombus (ISPAT) or low-flow stasis artifact (LFSA) within the pulmonary arteries can mimic acute PE. The proposed mechanism for ISPAT is chronic stasis due to abnormal perfusion in areas of parenchymal lung disease leading to in situ thrombosis. Similarly, LFSA occurs when stasis leads to persistent visualization of intravenous contrast which is then mistaken for thrombus. The clinical scenarios in which ISPAT and LFSA develop are not yet fully defined. We report here a series of patients with parenchymal lung disease leading to ventilation-perfusion mismatch who likely had ISPAT or LFSA and not acute PE. Our aim is to further define parenchymal lung disease as a subgroup of patients who are at high risk for ISPAT. Cases initially diagnosed as acute PE leading to activation of the UCLA pulmonary embolism response team (PERT) were reviewed. Inclusion criteria were all cases of PE that led to PERT activation. Inclusion criteria included absence of DVT, previously diagnosed pulmonary disease, and presence of thrombus only in areas of abnormal parenchyma. Cases were reviewed with radiology to identify cases in which ISPAT was the likely diagnosis, and five representative cases were selected to be discussed. These cases were then analyzed qualitatively for commonalities which are described below. The five representative cases described represent patients with known chronic lung disease who were diagnosed and initially managed as acute PE but, on review, met criteria for either ISPAT or stasis artifact. These cases, which were all rediagnosed as ISPAT or LFSA, were all seen in the absence of DVT, with thrombus specifically located in areas of significant parenchymal lung disease with suspected decrease in ventilation and perfusion in these areas. ISPAT or LFSA have been described in the literature, though its presence specifically in parenchymal lung disease has yet to be described. The authors recommend considering this diagnosis in patient's diagnosed with acute PE when the following are present: significant parenchymal lung disease, absence of DVT, absence of thrombus in the areas of the lung relatively spared of parenchymal lung disease, and suspected baseline decrease in both ventilation and perfusion to the affected areas of the lung. As this phenomenon may be physiologically beneficial, the authors suggest that not all cases of ISPAT or LFSA need anticoagulation, and that treatment should be considered on a case-by-case basis.
Renal impairment is considered a contra-indication for lung (LTX) or combined heart-lung (HLTX) transplantation due to increased mortality. We hypothesized that renal impairment in pulmonary arterial hypertension (PAH) a...Renal impairment is considered a contra-indication for lung (LTX) or combined heart-lung (HLTX) transplantation due to increased mortality. We hypothesized that renal impairment in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) is the result of reduced cardiac output and should be partly reversible after LTX. We performed a retrospective analysis in 67 consecutive PAH and CTEPH patients who underwent (H)LTX, to investigate the postoperative evolution of renal function in function of baseline renal function using a mixed model effect test. Furthermore, we assessed potential predictors for postoperative renal dysfunction, renal replacement therapy (RRT) and mortality by multivariate analyses. Median baseline eGFR was 74 mL/min/1.73m². Fourteen patients were classified as KDIGO 3 preoperatively, 38 patients as KDIGO 2. Renal function significantly declined after 1 and 2 years in all patients. In patients with impaired renal function (KDIGO 2 and 3), we observed a significant improvement in eGFR 1 month after (H)LTX (= 0.02 and = 0.04, respectively). Baseline renal impairment ≤ 60 mL/min/1.73m² was associated with early RRT but not with further renal function deterioration, long-term RRT, or mortality. Age was a predictor of renal function decline and mortality. We conclude that renal function evolution can be biphasic after (H)LTX in PAH and CTEPH patients with baseline renal impairment, with initial improvement due to resolution of cardio-renal syndrome. Mild to moderate renal impairment was not significantly associated with renal deterioration or increased mortality.
Pulmonary endarterectomy (PEA) is the gold standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH). While balloon pulmonary angioplasty (BPA) is an emerging treatment for distal CTEPH, a standard the...Pulmonary endarterectomy (PEA) is the gold standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH). While balloon pulmonary angioplasty (BPA) is an emerging treatment for distal CTEPH, a standard therapeutic strategy for CTEPH with unilateral central lesions has yet to be established. Herein, we describe the successful treatment of a patient with CTEPH who underwent BPA for left distal lesions, followed by PEA for unilateral right central lesions, without serious complications. BPA before PEA may reduce perioperative complications by improving hemodynamics and contribute to a better clinical course by shortening deep hypothermic circulatory arrest time through reduction of the PEA treatment area. Depending on the anatomical characteristics of the lesions, this combination therapy should be discussed by a multidisciplinary CTEPH team.
To analyze the clinical characteristics and potential pregnancy outcomes of deceased pregnant women with pulmonary hypertension, we conducted a retrospective analysis of clinical data from 54 cases of pregnant women with...To analyze the clinical characteristics and potential pregnancy outcomes of deceased pregnant women with pulmonary hypertension, we conducted a retrospective analysis of clinical data from 54 cases of pregnant women with pulmonary hypertension at The Third Affiliated Hospital of Guangzhou Medical University from May 2009 to February 2022. The results demonstrated that (1) Among 54 deceased pregnant patients with pulmonary hypertension (PH), 44 patients belonged to type 1, and 3, 2, and 5 patients belonged to type 2, type 4, and type 5, respectively. In type 1, 33 cases were secondary to congenital heart disease, with ventricular septal defect being common. (2) All 54 patients were diagnosed with PAH during pregnancy or postpartum. NYHA cardiac function grade II (10 cases), Ⅲ (18 cases), Ⅳ (26 cases), heart disease pregnancy risk levels were all Ⅳ. (3) Of the 54 patients, 7 patients had no data on the time of death, and most of them (37 patients) died within 1 week postpartum. (4) These 9 patients with mild PAH death during pregnancy had lower ejection fraction, higher rates of postpartum hemorrhage, left heart failure, systemic failure, arrhythmia, gestational diabetes, and infection than those with moderate or severe PAH death during pregnancy. As a consequence, deaths in pregnancies complicated with PH were mostly caused by PAH crisis, total heart failure, multiple organ failure, Eisenmenger syndrome and maternal perinatal mortality was high. The primary causes of mortality in pregnant women with mild PH include heart failure, respiratory failure, and arrhythmia etc. Patients experiencing complications such as postpartum hemorrhage, left ventricular dysfunction, multiorgan failure, arrhythmia, gestational diabetes mellitus, and infection may have a poorer prognosis.
Pulmonary arterial hypertension (PAH) is a common condition among women of childbearing age (WCBA) and is associated with adverse outcomes during pregnancy. However, there is currently a lack of studies that provide a de...Pulmonary arterial hypertension (PAH) is a common condition among women of childbearing age (WCBA) and is associated with adverse outcomes during pregnancy. However, there is currently a lack of studies that provide a detailed epidemiological characterization of this condition. This study aimed to delineate the global burden of pulmonary arterial hypertension (PAH) among women of childbearing age (WCBA) from 1990 to 2021. We utilized the Global Burden of Disease Study 2021 to estimate the prevalence, incidence, mortality, and disability-adjusted life years (DALYs) for PAH among WCBA across 204 countries and territories from 1990 to 2021. Sociodemographic index (SDI) was used to assess the impact of socioeconomic development on PAH burden. In 2021, global estimates revealed 46,630 prevalent cases of PAH, resulting in 8532 new cases, 1777 deaths and 103,151 DALYs. Globally, the age-standardized prevalence, incidence, mortality, and DALY rates for PAH in 2021 stood at 2.35, 0.43, 0.09, and 5.26 per 100,000 population, respectively. In 2021, Switzerland exhibited the highest age-standardized prevalence rate (7.47/100,000). The highest age-standardized incidence rate was observed in Zambia (0.96/100,000). Mauritius reported the highest age-standardized mortality (0.72/100,000) and DALY rates (40.42/100,000), contrasting sharply with Moldova's lowest rates (0.00/100,000 and 0.51/100,000, respectively). At the regional level, the relationship between the SDI and age-standardized prevalence rates for PAH exhibited an approximate V-shaped pattern. The systematic analysis of PAH burden among WCBA underscores the disease's significant global impact and the necessity for continued research and tailored public health strategies, calling for enhanced awareness, improved diagnostics, and more effective treatment modalities, particularly in resource-constrained settings.
Chronic obstructive pulmonary disease (COPD) is frequently accompanied by abnormalities of the pulmonary vasculature. This vasculopathy spans the spectrum from mild vascular dysfunction to pulmonary hypertension, which o...Chronic obstructive pulmonary disease (COPD) is frequently accompanied by abnormalities of the pulmonary vasculature. This vasculopathy spans the spectrum from mild vascular dysfunction to pulmonary hypertension, which on rare occasions can be severe. Given the worldwide prevalence of COPD, it is conceivable that the morbidity and mortality associated with pulmonary vascular dysfunction have been vastly underappreciated, especially in countries and regions where the infrastructure and resources to define the magnitude of the problem are often limited. This article reflects deliberations of the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative (PVRI IDDI) Group 3 Pulmonary Hypertension Workstream on the role of the pulmonary vasculature in COPD. In this introductory paper, we lay the foundation for forthcoming papers by our group, with our ultimate goals being to increase awareness and encourage more research, including clinical trials, to address this unmet need.
This exploratory analysis assessed whether plasma biomarkers predict the response to switching from phosphodiesterase type 5 inhibitors (PDE5is) to the soluble guanylate cyclase stimulator riociguat in patients with pulm...This exploratory analysis assessed whether plasma biomarkers predict the response to switching from phosphodiesterase type 5 inhibitors (PDE5is) to the soluble guanylate cyclase stimulator riociguat in patients with pulmonary arterial hypertension. Selected biomarkers at baseline and their changes to Week 24 were evaluated in patients with and without a favorable response to riociguat in two trials: RESPITE, in which patients with an inadequate response to PDE5i were switched to riociguat; and REPLACE, in which patients at intermediate risk of 1-year mortality despite a PDE5i were randomized to remain on PDE5i or were switched to riociguat. A response was defined as absence of clinical worsening and at least two of the following criteria: 6-min walk distance increase by 10% or ≥ 30 m, World Health Organization functional class I/II, or -terminal prohormone of brain natriuretic peptide reduction of ≥ 30% at Week 24. In REPLACE, responders had significantly higher baseline cyclic guanosine monophosphate (cGMP) and significantly lower baseline asymmetric dimethylarginine, and growth/differentiation factor 15 (GDF-15) than nonresponders. In RESPITE, responders had lower baseline GDF-15 than nonresponders, and nonresponders showed a significantly greater decrease in cGMP than responders. No baseline threshold value of any biomarker provided a good likelihood of predicting the response to riociguat. Overall, the biomarkers evaluated did not help to identify patients who were more likely to respond to switching from PDE5is to riociguat.
Pulmonary hypertension in COPD patients and identifying accompanying factors in clinical decisions are very important. The current study aimed to determine the relationship between phenotypic and echocardiographic findin...Pulmonary hypertension in COPD patients and identifying accompanying factors in clinical decisions are very important. The current study aimed to determine the relationship between phenotypic and echocardiographic findings with the help of pulmonary artery pressure in patients with COPD. In this study, 100 COPD patients referred to a specialized clinic participated in the study. The prevalence of pulmonary hypertension (PH) and its relationship with phenotypic criteria, spirometry, laboratory findings, and echocardiography in COPD patients were investigated. Among the COPD patients, 76 cases were classified as having chronic bronchitis, nine as having emphysema, and 15 were classified as mixed phenotype by Chest high-resolution computed tomography (HRCT) methods. The prevalence of PH in the study was 36% (moderate 24% and severe 12%). The average pulmonary arterial pressure (PAP) was significantly difference in different phenotypes ( < 0.001). Heart rate and PAP significantly increase in mixed phenotypes = 0.029 and = 0.002, respectively. Also, the modified Medical Research Council scale (mMRC) and BODE index were significantly increase in mixed phenotypes of COPD patients ( < 0.05 to < 0.001). Compared the factors related to PH in different phenotypes of patients with COPD indicated that PH are related with heart rate, PCO2, creatinine, triglycerides, 6MWT and BODE Index.