Identification and functional analysis of biallelic loss-of-function variants of WNT7B in a Chinese family affected with PDAC syndrome.

The clinical integration of four-dimensional flow magnetic resonance imaging (4D-flow MRI) for cardiovascular assessment remains a challenge due to the expertise and resources required for analysis, and there is no large-scale systematic validation of the technique. We aim to evaluate the bulk flow measurements in the large vessels using 4D-flow MRI compared with conventional cardiac MRI in a large cohort as part of the prospective 4DCarE study. We conducted a large cohort prospective study to compare the bulk flow measurements in the ascending aorta and main pulmonary artery using 4D-flow and 2D phase contrast (2D-PC) MRI and analysed the 4D-flow measurements using two different commercially available software packages. A total of 500 participants with a normal or pathological cardiac history were evaluated. Within the limitations of technical differences, 4D-flow demonstrated clinically consistent and reliable measurements compared with 2D-PC, although 4D-flow measurements showed a systematic bias towards undermeasurement compared with 2D-PC, irrespective of software used. Both 4D-flow and 2D-PC demonstrated good internal consistency for normal and pathological cases. The incorporation of artificial intelligence in the image annotation process showed early promises in reducing time and labour intensity and potentially improving the clinical integration of 4D-flow MRI, however, the technology remains nascent, and further development and validation are required. Bulk flow measurements in the large vessels were grossly clinically consistent between 4D-flow and 2D-PC in this large cohort study, although 4D-flow showed systematic undermeasurement compared with 2D-PC.

Nonthrombotic pulmonary artery embolism (NTPE) involves occlusion of pulmonary arteries by nonthrombotic material, such as septic emboli, tumor cells, fat, air, or foreign substances. NTPE is less common than thrombotic pulmonary embolism (PE) and may be misdiagnosed as PE. Although the clinical manifestation mimics that of PE, NTPE has distinct pathophysiologic mechanisms that necessitate different management. Diagnosis requires a high index of clinical suspicion and knowledge of imaging findings. The authors provide an overview of the various causes of NTPE, including infectious, neoplastic, iatrogenic or exogenous, and miscellaneous entities, and highlight their key imaging findings. Early and accurate diagnosis is essential for appropriate management. ©RSNA, 2026.

Association of pulmonary arterial end-diastolic forward flow and right heart remodeling in dogs with congenital pulmonic stenosis and precapillary pulmonary hypertension.

Silencer-regulated circLSM14A inhibits autophagy of pulmonary artery smooth muscle cells through parental protein LSM14A.

Therapeutic venesection is a cornerstone in the management of polycythaemia to reduce hyperviscosity and thrombotic risk. However, its application in patients receiving anticoagulation, particularly heparin, is complex due to the potential for bleeding complications. The current case report describes a 40-year-old male who presented with progressive breathlessness, fatigue, and pedal oedema. Laboratory investigations revealed polycythaemia with elevated haemoglobin and haematocrit levels. The patient was diagnosed with acute pulmonary thromboembolism and severe pulmonary artery hypertension. He was initiated on unfractionated heparin therapy. Due to persistent symptoms and hyperviscosity, therapeutic venesection was performed after temporarily withholding heparin. A total volume of 450 mL of blood was removed. The procedure was well tolerated, with stable vital signs, and the patient showed symptomatic improvement following venesection. The present case highlights the therapeutic benefit and safety of venesection in a patient with polycythaemia and pulmonary thromboembolism receiving anticoagulation. Meticulous risk assessment, temporary cessation of anticoagulation at the time of venesection, and strict procedural monitoring were critical in preventing complications. A multidisciplinary approach ensured optimal patient outcomes. The novelty of the current case lies in the successful integration of therapeutic venesection in a high-risk anticoagulated patient, guided by an individualized risk-benefit assessment and coordinated multidisciplinary management to optimise safety and outcomes in complex thrombotic scenarios.

Tricuspid annular plane systolic excursion (TAPSE) to pulmonary artery systolic pressure (PASP) is an established prognostic marker in tricuspid regurgitation (TR). Right ventricular free wall longitudinal strain (RVFWS)/PASP has been proposed as more sensitive prognostic markers than TAPSE/PASP in patients with TR, yet its role in patients undergoing transcatheter tricuspid valve repair (TTVr) remains unclear. The objective of the study was to evaluate and compare the prognostic value of RVFWS/PASP and TAPSE/PASP with two different vendors in symptomatic TTVr cohorts. TTVr-treated patients across three centers (2017-2023) were included. RVFWS was assessed using two vendors (A = GE EchoPAC and B = Tomtec). The primary endpoint was a composite of all-cause mortality or the first heart failure hospitalization at 2-years. Prognostic performance was assessed using Cox regression, C-statistics, and Kaplan-Meier analysis. A total of 349 patients were included (137 vendor A and 212 vendor B). Patients in vendor B were older (83 vs. 80; p < 0.001), more symptomatic (New York Heart Association III/IV: 95.8 vs. 81.7%; p < 0.001), had lower 6-minute walk distance (218 vs. 282 m; p < 0.001), and greater right heart dilation, despite similar TR severity. RVFWS was higher in vendor B (23.0 vs. 18.0%, p < 0.001). RVFWS/PASP emerged as an independent predictor of the primary endpoint only in vendor A (hazard ratio per %/mmHg increase: 0.07, 95% CI: 0.01-0.46; p = 0.005), whereas TAPSE/PASP consistently showed independent prognostic association in both cohorts. Discrimination of both indices was not significantly different. Receiver operating characteristics-derived cutoffs identified patient subgroups with markedly different 2-year outcomes in both cohorts. Both RVFWS/PASP and TAPSE/PASP provided modest prognostic value after TTVr, with TAPSE/PASP demonstrating consistent independent association across vendors.

Unilateral absence of pulmonary artery (UAPA) is an uncommon condition with a prevalence of 1 in 200,000 young adults, which is characterised by congenital absence of either the left or right pulmonary artery and may be associated with or without other cardiac diseases. While the UAPA declares itself in infancy when associated with other congenital anomalies, patients with isolated UAPA usually remain asymptomatic and present in adulthood with symptoms such as dyspnoea, haemoptysis, recurrent infections, etc. We encountered an adult male patient who presented with haemoptysis and was diagnosed on further examination to have an absent right pulmonary artery. Considering the rarity and non-specific presentation of isolated UAPA, this case is being reported.

BackgroundPrimary tumors of the pulmonary artery are rare and frequently misdiagnosed as thromboembolic disease. Benign chondroid tumors arising from this location are exceptionally uncommon.Case DescriptionA 23-year-old man presented with exertional dyspnea and syncope. Imaging revealed a well-defined mass occupying the main pulmonary artery. Positron emission tomography/computed tomography showed no metabolic activity. The mass was completely excised under cardiopulmonary bypass. Histopathological examination demonstrated mature hyaline cartilage with low proliferative activity, consistent with a benign chondroid tumor. The postoperative course was uneventful, and no recurrence was observed at 12-month follow-up. This case highlights a rare benign etiology of pulmonary artery obstruction. Surgical resection remains the definitive diagnostic and therapeutic approach in selected patients with atypical intraluminal pulmonary artery masses.

Introduction: Prolonged mechanical ventilation (PMV) is a postoperative burden in single ventricle physiology (SVP) pediatric patients undergoing pulmonary artery (PA) banding. Predicting the optimal band tightness and its postoperative impact remains challenging, particularly in low- and middle-income countries, as evidence linking risk factors to PMV following PA banding is still limited. This study aimed to identify perioperative risk factors associated with PMV following PA banding in pediatric patients with SVP. Methods: This retrospective, single-center cohort study included 101 children with SVP who underwent PA banding between 2017 and 2023. Binomial logistic regression models were used to examine the association of perioperative risk factors and PMV (>126 h). Secondary outcomes included in-hospital mortality, postoperative complications, and PA rebanding. A P-value of < .05 was considered statistically significant. Results: Prolonged MV occurred in 31.7% (32/101) of patients. A higher peak right ventricle-pulmonary artery (RV-PA) pressure gradient was associated with lower odds of postoperative prolonged MV (OR = 0.894, P = .018). Significantly lower PMV risk was associated with RV-PA gradient values above the cut-off value of 39.5 mm Hg (AUC 0.665, 95% CI 0.538-0.792, P = .012, Youden index 0.387). Nevertheless, abnormally low postoperative mean arterial pressure (MAP) was independently associated with PMV (P = .038). Conclusions: Peak RV-PA gradient > 39.5 mm Hg and stable postbanding MAP help reduce PMV risk in children with SVP undergoing PA banding. Incorporating bodyweight and oxygenation status in prebanding assessment may reduce risk of postoperative mortality. These findings offer guidance for integrated perioperative assessment, particularly in resources-limited centers.

Lung transplantation remains the primary option to treat end-stage lung disease, and treatments aiming to improve graft quality are necessary. Exvivo lung perfusion (EVLP) is a strategy that allows the organ to be assessed and reconditioned before transplantation. Treatment of the donor with a combination of 17β-estradiol (E2) and methylprednisolone (MP) has been shown to improve lung quality after brain death (BD). All considered, this study aimed to investigate E2 and MP association during EVLP. Males and females Wistar rats underwent BD induction and were maintained for 4 h. Naive animals were used as control. After BD, the pulmonary artery was cannulated, the heart-lung en bloc was collected, submitted to cold ischemia (1 h) and then placed in an EVLP system (4 h). Perfusion solution was home-made perfusate added or not with the treatment (T: MP, 40 mg; E2: 5 μg/mL). Groups were defined as male and female rats, divided as follows: BD (without perfusion), EVLP (without treatment), EVLP+Treat (with treatment). Male EVLP+Treat presented increased dynamic and static compliance, increased paO2, and reduced elastance. Treated males also presented reduced iNOS and MPO and increased perfusion flow. Both female perfused groups presented reduced MPO and adhesion molecules. Female EVLP+Treat also presented increased flow. No difference in lung function was observed in females. Our results point to a positive effect in the combined use of E2 and MP during EVLP by improving lung function and decreasing inflammation, especially in males.

Platelet gene signatures detecting pulmonary artery stenosis in patients with pulmonary hypertension.

Objective: To explore the clinical features, diagnosis, and treatment progress of angiomatoid fibrous histiocytoma (AFH) primary to the pulmonary artery. Methods: A retrospective analysis was conducted on one case of AFH primary to the pulmonary artery in a middle-aged female. Databases including SinoMed, the Wanfang Data Knowledge Service Platform, and the China National Knowledge Infrastructure (CNKI) were searched using the keywords (angiomatoid fibrous histiocytoma) and (pulmonary artery). Meanwhile, PubMed, Embase, the Cochrane Library, and Web of Science were searched using the keywords "angiomatoid fibrous histiocytoma" and "pulmonary artery." The search was updated to April 2025. Results: The patient was admitted to our hospital in February 2025 due to symptoms such as chest tightness and dizziness, and a space-occupying lesion of the pulmonary artery was detected. Postoperative histopathological examination confirmed the diagnosis of pulmonary artery AFH. Only one relevant article was retrieved from domestic databases and five from foreign databases. A total of seven patients were included (including the current case), with four males and three females, aged 20-76 years (mean, 54.57±18.18 years). The lesions were located in the left main pulmonary artery (three cases), right main pulmonary artery (two cases), right upper pulmonary artery (three cases), and right interlobar pulmonary artery (one case). D-dimer levels were elevated in two cases, normal in three cases, and unknown in two cases. PET-CT showed significantly increased SUVmax in four cases and was unknown in three cases. EWSR1 gene translocation was detected in five cases, absent in one case, and unknown in one case. Surgical procedures included extended local resection of the pulmonary artery (three cases) and partial or total pneumonectomy (four cases). No recurrence or metastasis was observed in five patients, and the outcomes of two patients were unknown. Conclusion: AFH primary to the pulmonary artery is extremely rare. Clinically, it needs to be mainly differentiated from pulmonary thromboembolism and pulmonary artery intimal sarcoma, and clinicians in respiratory medicine and vascular intervention departments should raise awareness of this disease. In the differential diagnosis from pulmonary thromboembolism, clinical manifestations and laboratory tests (e.g., D-dimer) lack specificity, while imaging examinations such as CTPA and PET-CT are effective auxiliary methods. Definitive diagnosis still relies on histopathological examination, and EWSR1 molecular detection is an important basis for pathological confirmation. Pulmonary artery resection combined with artificial vascular replacement is an effective treatment, and long-term follow-up is necessary.

Objective: This study aims to evaluate the clinical effectiveness of a modified balloon pulmonary angioplasty(BPA) procedure in the treatment of chronic thromboembolic pulmonary hypertension. Methods: We conducted a retrospective analysis of clinical data from patients diagnosed with chronic thromboembolic pulmonary hypertension who underwent Modified BPA at the Department of Cardiovascular Medicine in Xiamen Hospital of Traditional Chinese Medicine between January 2021 and May 2024. The study comprised 21 patients, including 5 males and 16 females, who successfully underwent the scheduled six BPA surgical sessions, totaling 126 individual cases. Results: All surgeries were guided by pressure guidewires. Three patients exhibited mild hemoptysis during the procedure, while two experienced mild pulmonary edema in the postoperative phase. There were no fatalities. Compared to the baseline, significant improvements were observed in indicators such as mean pulmonary artery pressure, pulmonary vascular resistance, and cardiac index following the surgical treatment course for all patients. These differences were statistically significant (P<0.001) as measured by right heart catheterization. Three months post-treatment, there was a marked improvement in cardiac function classification (WHO FC), 6-minute walking distance, and NT-proBNP levels compared to the baseline, with these differences also being statistically significant (P<0.001). Additionally, significant enhancements were noted in the right atrial area, estimated pulmonary artery systolic pressure, and tricuspid annular plane systolic excursion (TAPSE), all of which exhibited statistically significant differences (P<0.001). Conclusions: The modified BPA procedure significantly improves the structure of the right heart, cardiac function, and hemodynamics in patients suffering from chronic thromboembolic pulmonary hypertension (CTEPH). This intervention is not only safe but also highly effective, positioning it as an exceptional therapeutic option for patients with CTEPH.

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.

Acute pulmonary thromboembolism (PTE) is a clinical emergency caused by thrombus obstruction of the pulmonary artery. The mortality rate of high-risk PTE patients without timely intervention is as high as 10%-30%, while standardized interventional therapy can significantly reduce the mortality rate to about 3%. Although interventional therapy has been widely used in the treatment of PTE patients, there is currently a lack of standardized operating procedures for this technique, and there are significant differences among different medical institutions and operators. In order to further improve the level of interventional therapy for PTE in China and promote the standardization of interventional therapy for PTE, the Pulmonary Embolism and Pulmonary Vascular Disease Group of the Respiratory Diseases Branch of the Chinese Medical Association and the Pulmonary Embolism and Pulmonary Vascular Disease Working Group of the Respiratory Physicians Branch of the Chinese Medical Doctor Association jointly organized experts in related fields in China. Based on the latest evidence-based medical evidence and combined with China's clinical practice, they focused on the patient selection, operating procedures, and peri-operative management strategies for interventional therapy of acute PTE. After multiple rounds of discussion, they jointly formulated the "Expert Consensus on Operating Procedures for Interventional Therapy of Acute Pulmonary Thromboembolism".Recommendation 1: Interventional therapy for acute PTE should be based on risk stratification. Intermediate-risk and high-risk patients may be considered for interventional therapy under the decision-making of multidisciplinary discussion.Recommendation 2: Interventional therapy has unique advantages in the treatment of acute PTE patients, but local technical conditions should be considered, including factors such as the availability of medical devices and the experience of operators.Recommendation 3: For trans-catheter thrombus removal in acute PTE, dedicated devices are recommended to improve efficiency and safety.Recommendation 4: Interventional therapy is recommended in the following situations: high-risk acute PTE with contraindications to or failure of thrombolytic therapy; intermediate-high-risk acute PTE with contraindications to or failure of thrombolytic therapy; and intermediate-high-risk acute PTE with hemodynamic deterioration during anticoagulant therapy.Recommendation 5: The multidisciplinary teams required for interventional therapy of acute PTE include the Department of Respiratory and Critical Care Medicine, Intensive Care Unit (ICU), Department of Cardiovascular Medicine and Surgery, Department of Thoracic Surgery, Department of Radiological Interventions, etc.Recommendation 6: Pulmonary angiography should be performed before interventional therapy to clarify the location and degree of pulmonary thromboembolism, guide interventional therapy, and improve efficiency.Recommendation 7: During the operation, blood loss should be minimized, vital signs such as blood pressure and heart rate should be monitored in real time, and the patient's vital signs and subjective feelings should be paid attention to avoid serious complications such as hemorrhagic shock (It is recommended to control blood loss below 200-300 ml).Recommendation 8: The goals of interventional therapy for acute PTE are to improve oxygenation and hemodynamics, such as decreased heart rate, increased systemic blood pressure, reduction in vasoactive medications, and increased oxygen saturation, etc.Recommendation 9: Heparinization is recommended during the operation (unless strictly contraindicated), and anticoagulation should be continued after the operation.

Dysfunction of pulmonary artery endothelial cells (PAECs) contributes to the pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH). However, the role of mitochondrial metabolism in this process remains unclear. The present study evaluated whether the tetrameric form of pyruvate kinase muscle isoform 2 (PKM2) regulates PAEC mitochondrial metabolism through peroxisome proliferator-activated receptor gamma coactivator 1 alpha (PGC-1alpha) and mitochondrial transcription factor A (mtTFA), thereby influencing arterial intimal remodeling in CTEPH. A CTEPH rat model was established by repeated injections of autologous thrombi. Activation of PKM2 tetramer expression was achieved through synthetic pyruvate kinase M2 activator (TEPP-46) administration. Pulmonary artery pressure (PAP), thrombus pathology, and protein expression levels of PKM2, mtTFA, and PGC-1alpha were assessed. Plasma lactate concentrations and tumor necrosis factor alpha (TNF-alpha) levels were measured. Rats with CTEPH demonstrated thrombotic obstruction, elevated PAP, and reduced expression of the PKM2 tetramer, mtTFA, and PGC-1alpha. Treatment with TEPP-46 was associated with a reduction in thrombus burden, lower PAP, and restoration of mitochondrial protein expression, accompanied by decreased lactate concentrations and TNF-alpha levels. In the CTEPH rat model, increased inflammation and elevated lactate concentrations were observed, along with decreased expression of mtTFA and PGC-1alpha in the pulmonary artery intima, which is indicative of mitochondrial dysfunction. The PKM2 tetramer may play a role in modulating PAEC mitochondrial function, reducing pulmonary artery pressure, and improving pulmonary arterial intimal remodeling in CTEPH. Key words Chronic thromboembolic pulmonary hypertension " Lactic acid " Mitochondrial transcription factor A " Peroxisome proliferator-activated receptor gamma coactivator 1alpha " Pyruvate kinase muscle.

Pulmonary Hypertension Associated With Left Heart Disease: Challenges, Emerging Strategies, and Future Directions.

Hiatal hernia (HH), a common digestive disease, may impact the respiratory system, but systematic evaluation of its effect on cardiopulmonary function and associations with clinical indicators remains insufficient. This retrospective study included 248 HH patients (118 surgical [SHH], 130 non-surgical [NHH]) and 516 healthy controls (HC) from The First Affiliated Hospital of Zhejiang University School of Medicine (January 2021-December 2024).Data on basic information, imaging, pulmonary function, echocardiography, and reflux symptoms were collected. Differences in cardiopulmonary function among groups were compared through multi-factor adjustment analysis, and related influencing factors were explored. HH patients had higher pulmonary infection risk, poorer ventilatory/small airway/diffusing function, and wider pulmonary artery (PA) diameter. NHH patients were older, had larger hernia sacs, and worse cardiopulmonary function but fewer reflux symptoms.The differences in cardiopulmonary function among the above groups weakened after excluding confounding factors such as age. Advanced age and large hernia sac (not reflux) correlated with impaired cardiopulmonary function. 81.2% of SHH patients had improved postoperative respiratory symptoms. HH is associated with cardiopulmonary dysfunction, manifested by increased risk of pulmonary infection, restrictive ventilatory dysfunction, decreased diffusing capacity, and widened PA diameter. These associations are not significantly associated with reflux and mainly related to age and hernia sac size. This study has limitations due to its retrospective design and can only provide preliminary data reference for exploring the cardiopulmonary function characteristics of HH and their related influencing factors.

Dysfunction of pulmonary artery endothelial cells (PAECs) contributes to the pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH). However, the role of mitochondrial metabolism in this process remains unclear. The present study evaluated whether the tetrameric form of pyruvate kinase muscle isoform 2 (PKM2) regulates PAEC mitochondrial metabolism through peroxisome proliferator-activated receptor gamma coactivator 1 alpha (PGC-1α) and mitochondrial transcription factor A (mtTFA), thereby influencing arterial intimal remodeling in CTEPH. A CTEPH rat model was established by repeated injections of autologous thrombi. Activation of PKM2 tetramer expression was achieved through synthetic pyruvate kinase M2 activator (TEPP-46) administration. Pulmonary artery pressure (PAP), thrombus pathology, and protein expression levels of PKM2, mtTFA, and PGC-1α were assessed. Plasma lactate concentrations and tumor necrosis factor alpha (TNF-α) levels were measured. Rats with CTEPH demonstrated thrombotic obstruction, elevated PAP, and reduced expression of the PKM2 tetramer, mtTFA, and PGC-1α. Treatment with TEPP-46 was associated with a reduction in thrombus burden, lower PAP, and restoration of mitochondrial protein expression, accompanied by decreased lactate concentrations and TNF-α levels. In the CTEPH rat model, increased inflammation and elevated lactate concentrations were observed, along with decreased expression of mtTFA and PGC-1α in the pulmonary artery intima, which is indicative of mitochondrial dysfunction. The PKM2 tetramer may play a role in modulating PAEC mitochondrial function, reducing pulmonary artery pressure, and improving pulmonary arterial intimal remodeling in CTEPH.