Objective: To draw attention to the diagnosis of active Takayasu arteritis (TAK) in middle-aged and elderly patients and to analyze the clinical characteristics of this population. Methods: Clinical data of 106 patients newly diagnosed with TAK at Beijing Chao-Yang Hospital, Capital Medical University, between 2017 and 2025 were retrospectively collected; 11 patients aged over 50 years (2 men and 9 women) were enrolled in this study. Onset characteristics, medical history, clinical manifestations, laboratory findings, and imaging features were collected and analyzed. Disease activity was evaluated, treatment regimens and prognosis were recorded, and all patients underwent at least one follow-up. Results: Among patients first diagnosed with TAK at this single center, middle-aged and elderly patients with active TAK accounted for 10.40% (11/106) during the same period, with a median age at diagnosis of 61 years. Among them, 6 patients (6/11) were over 60 years old. The median time from symptom onset to diagnosis was 13 months (interquartile range: 12, 36 months). Although all 11 patients had multiple arterial involvements, none underwent complete evaluation of all arteries covered by the international criteria. Among the 8 patients who underwent CT pulmonary angiography (CTPA) or chest contrast-enhanced CT, all showed pulmonary artery luminal stenosis. All 8 patients who underwent contrast-enhanced magnetic resonance pulmonary angiography (MRPA) exhibited pulmonary artery wall thickening with enhancement and luminal stenosis. Of the 9 patients who underwent PET-CT, 7 showed pulmonary artery hypermetabolism. Among the 4 patients with elevated levels of multiple autoantibodies, 2 were male (2/2) and 2 were female (22.2% of female patients, 2/9). Moreover, both male patients had severe TAK-related complications (one with an abdominal aortic aneurysm and the other with pulmonary hypertension). Conclusion: Active TAK can occur in individuals over 60 years of age and the disease may be more severe in middle-aged and elderly male TAK patients. Middle-aged and elderly patients with active TAK require comprehensive evaluation of arterial involvement with modalities such as ultrasound, so as to enhance the capability of clinical differential diagnosis. For patients presenting primarily with respiratory symptoms, CTPA and MRPA should be performed to clarify pulmonary artery involvement in TAK.

This case report presents a young female patient whose chief complaint was exertional dyspnea. Right heart catheterization confirmed severe pulmonary hypertension. Concurrently, chest CT showed atypical pulmonary parenchymal and interstitial lesions, and pulmonary function tests indicated severe impairment of diffusion capacity. To clarify whether an association existed between the lung disease and pulmonary hypertension, transbronchial lung cryobiopsy was performed, which revealed multinucleated giant cell aggregates within the alveolar spaces, with cholesterol clefts in the cytoplasm of some of them. Together with a 1-year history of pigeon exposure, the final pulmonary diagnosis was extrinsic allergic alveolitis (hypersensitivity pneumonitis).With respect to the pulmonary hypertension, genetic testing showed no significant abnormalities, thereby ruling out heritable pulmonary hypertension. After oral imatinib combined with macitentan was administered, the patient's pulmonary artery pressure gradually decreased and her symptoms recovered well; however, follow-up chest CT and pulmonary function tests showed no apparent improvement. Because the elevated pulmonary artery pressure was inconsistent with the pulmonary disease, the patient was ultimately diagnosed with idiopathic pulmonary hypertension complicated by allergic alveolitis.

Identification of key driver genes in idiopathic pulmonary arterial hypertension by single-cell RNA sequencing and experimental validation.

A hallmark of pulmonary hypertension (PH) is the progressive increase in pulmonary arterial resistance caused by impaired pulmonary artery (PA) relaxation and vascular remodeling. Salusin-α, a cardiovascular-active peptide, has been implicated in several cardiovascular diseases; however, its role in PH remains undefined. PAs were isolated from rats for isometric tension recording to assess relaxation. PA remodeling was quantified using histological morphometric analysis. Right ventricular pressure and hypertrophy were measured to evaluate PH severity. Salusin-α levels were significantly decreased in both plasma and PAs from PH rats compared with controls. Both acute and chronic salusin-α administration improved endothelium-dependent and -independent PA relaxation, increased endothelial nitric oxide synthase activity and nitric oxide levels in PA endothelial cells, enhanced Nrf2 (nuclear factor erythroid 2-related factor 2) and superoxide dismutase expression and activity in PA smooth muscle cells, and decreased NAD(P)H oxidase expression, activity, and reactive oxygen species levels. Salusin-α also inhibited PA smooth muscle cell proliferation and migration, attenuated pulmonary vascular remodeling, and decreased right ventricular pressure, hypertrophy, and mortality in PH rats. Neutralizing endogenous salusin-α exerted opposite effects. Pharmacological inhibition demonstrated that endothelial nitric oxide synthase and superoxide dismutase contributed to the protective actions of salusin-α, whereas NAD(P)H oxidase inhibition or reactive oxygen species scavenging prevented the effects of salusin-α neutralization. Salusin-α improves endothelium-dependent vasodilation via endothelial nitric oxide synthase mediated nitric oxide release in PA endothelial cells, and promotes endothelium-independent vasodilation while attenuating pulmonary vascular remodeling via inhibition of NAD(P)H oxidase-reactive oxygen species signaling and activation of the Nrf2/superoxide dismutase signaling. Restoring reduced salusin-α may present a novel therapeutic strategy for PH treatment.

Inflachromene attenuates monocrotaline-induced pulmonary arterial hypertension by suppressing the HMGB1-TLR4/RAGE-NF-κB signaling pathway.

Nasal septal deviation (NSD) is a common cause of chronic upper airway obstruction (UAO), and surgical correction via nasal septoplasty results in improvements in airflow. Multiple studies have been conducted to assess possible relationships between chronic UAO (including due to NSD) and cardiorespiratory function (such as pulmonary artery pressures). The objective of this scoping review is to identify and examine the current evidence on the potential relationship between nasal septal deviation, estimated mean pulmonary artery pressure (mPAP), and estimated pulmonary artery systolic pressure (ePASP) in patients who undergo nasal septoplasty. PubMed, EMBASE, Ovid. A scoping review was performed in accordance with the PRISMA Extension for Scoping Reviews guidelines. Two independent reviewers screened 411 articles; 9 before-after studies met eligibility criteria for review inclusion. There was a total of 458 study participants, of which 395 underwent septoplasty and 63 were matched controls. All studies reported a statistically significant decrease between the pre-operative and post-operative estimated mPAP (p < 0.05) and/or ePASP (p < 0.001) in subjects who underwent nasal septoplasty. Two studies included control participants and reported a statistically significant difference between the pre-operative estimated mPAP of patients with NSD and controls (p < 0.001). This scoping review highlights the current evidence on the potential relationship between NSD, estimated mPAP, and ePASP in patients who undergo nasal septoplasty for NSD. Further studies of higher statistical and methodological quality are required to assess for cause-and-effect relationships and examine the potential link between chronic UAO and cardiorespiratory function.

Large conductance calcium-activated potassium channels (BKCa) play an important role in the regulation of vascular tone. However, the properties of BKCa channels in smooth muscle of pulmonary arteries are poorly understood. Previous experimental studies demonstrated that pulmonary hypoxic vasoconstriction as a normal physiological response to decreased oxygen levels was impaired in diabetic animals due to abnormal activation of BKCa channels. The aim of this study was to identify mechanisms of diabetes-induced activation of BKCa channels in freshly isolated smooth muscle cells from rat pulmonary arteries. Type 1 diabetes was induced by streptozotocin (STZ). Whole-cell potassium currents were recorded using the patch-clamp method. Expression levels of BK-α and BK-β1 subunits were measured by real-time PCR. Our results demonstrate that the amplitude of whole-cell current through BKCa channels in rat pulmonary artery smooth muscle cells is significantly increased during STZ-induced diabetes without altering the expression of BK-α and BK-β1 subunits and channel calcium sensitivity. The slow component of BKCa current deactivation time constant and spontaneous transient outward current amplitude were increased in diabetic animals compared to healthy animals. We conclude that abnormal activation of the BKCa channel in pulmonary arterial smooth muscle during diabetes is associated with alterations in the local control mechanism of the BKCa pore-gate domain.

Interstitial Lung Disease with and without Mean Pulmonary Artery Pressure &gt;20 mmHg: Interim Results from the PHINDER Study

Impact of Continuous Allocation Score on Changes in Mean Pulmonary Artery Pressure Before Lung Transplantation

Pulmonary Hypertension: From Molecular Pathways to Patient Care.

Evidence guiding the optimal timing of mechanical thrombectomy for patients presenting with intermediate-risk pulmonary embolism (PE) is limited. We aimed to evaluate whether the timing of mechanical thrombectomy is associated with improved clinical outcomes in this patient population. This multicenter, retrospective cohort study was conducted at five large academic hospitals. In total, 290 patients presenting with intermediate-risk PE who were treated with mechanical thrombectomy were included for analysis. The primary outcome was in-hospital mortality. Using generalized estimated equation, we compared the odds of in-hospital mortality for patients undergoing early intervention (EI; mechanical thrombectomy < 12 hr after PE diagnosis) vs. those undergoing delayed intervention (DI; mechanical thrombectomy ≥ 12 hr after diagnosis) while comparing for PE severity and other confounders. None. EI was performed in 179 patients (61.7%), while 111 patients (38.3%) received DI. Unadjusted mortality did not differ significantly between groups (7.3% [13/179] vs. 10.8% [12/111]; p = 0.39). After adjusting for the Pulmonary Embolism Severity Index and Composite Pulmonary Embolism Shock scores, timing of intervention did not influence mortality (odds ratio, 1.80; 95% CI, 0.82-3.95; p = 0.14). However, patients in the EI group had greater reductions in in pulmonary artery systolic pressure (-25.8% [17.0] vs. -18.9% [17.1]; p = 0.020 and mean pulmonary artery pressure, -26.8% [17.7] vs. -20.2% [19.7]; p = 0.016) and lower rates of intubation (8.9% [16/179] vs. 18% [20/111]; p = 0.028). In patients presenting with intermediate-risk PE, timing of mechanical thrombectomy did not influence in-hospital mortality. EI may result in greater reductions in pulmonary artery pressures and decreased incidence of intubation compared with DI.

Borderline Pulmonary Artery Wedge Pressures and Their Association with Baseline Characteristics and Outcomes in Group 1 Pulmonary Arterial Hypertension: Results from the Pulmonary Hypertension Association Registry

Pulmonary arterial hypertension (PAH) is a syndrome characterized by elevated pulmonary artery pressure. We developed a population pharmacokinetic (PPK) model using combined plasma drug concentration data from Japanese paediatric (<15 years) and adult (≥15 years) patients with PAH to investigate the pharmacokinetic characteristics of PAH treatments (sildenafil, tadalafil, bosentan and ambrisentan). PPK analysis was conducted using a nonlinear mixed-effects modelling approach. A one-compartment model was selected as the structural model, and an exponential error model was used to describe interindividual variability. Individual patient clearance (CL), adjusted for bioavailability (F) using Bayesian estimation based on the PPK model, was used to estimate the steady-state plasma concentrations (Css) of the drugs in paediatric and adult patients receiving clinical dosages. PPK analysis was performed on data from 35 paediatric and 16 adult subjects to estimate pharmacokinetic parameters. The population mean CL/F values for sildenafil, tadalafil, bosentan and ambrisentan were estimated at 60.6, 1.76, 8.85 and 0.594 L/h, respectively. Covariates that improved model performance and were incorporated into the final model included the effect of bodyweight on the CL/F of each drug. The estimated Css values for all drugs were 26.0%-72.2% lower in paediatric than in adult patients. This study clarified the pharmacokinetic parameters and their variability for sildenafil, tadalafil, bosentan and ambrisentan using combined data from Japanese paediatric and adult patients with PAH. The results of Bayesian estimation suggest that at current clinical dosages, plasma concentrations of these drugs may be lower in paediatric than adult patients.

Impaired Lung BCAA Metabolism Promotes Ferroptosis and Resultant Pulmonary Arterial Hypertension-Associated Hepatopathy.

Household accidental injuries can occur via several mechanisms, but fatal penetration injuries are rare. A rare case of fatal penetration injury caused by a homemade pepper grinder is reported. The broken blades of a pepper grinder fatally injured a 72-year-old male. The blade fragments penetrated the individual's chest, injuring the aorta, the pulmonary artery and the lungs, resulting in hemorrhagic shock. The exact position of the blade was determined via postmortem computed tomography (PM-CT). This case demonstrates the injuring capacity of homemade rotating tools.

Predicting Pulmonary Artery - Pulmonary Capillary Wedge Pressure Decoupling from a Pulmonary Artery Pressure Sensor in HM3 LVAD Recipients- Analysis from the RAMP-IT-UP 3 Study

Prognostic value of TAPSE/sPAP in pulmonary arterial hypertension: a systematic review and meta-analysis.

Anomalous right coronary artery arising from the pulmonary artery (ARCAPA): an incidental diagnosis in a 3-year-old girl.

Agreement of minimally invasive pulse wave analysis with pulmonary artery and transpulmonary thermodilution cardiac output measurements in perioperative and intensive care medicine: a systematic review and meta-analysis.

Successful Lung Transplantation After Pulmonary Artery Rupture in End-Stage Pulmonary Arterial Hypertension: A Case Report