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Objectives: To investigate the potential utility of [18F]fibroblast activation protein inhibitor (FAPI) positron emission tomography/computed tomography (PET/CT) for evaluating pulmonary artery (PA) masses, and compare it with [18F]fluorodeoxyglucose (FDG) PET/CT. Methods: Participants with clinically suspected PA malignancy were prospectively enrolled and underwent dual-tracer PET/CT ([18F]FAPI-42 and [18F]FDG) imaging. Visual analysis and semi-quantitative parameters were compared between the two types of radiotracers. The tissue specimen underwent immunohistochemical staining to verify FAP expression in the tissue. Results: Thirty-three patients (18 males/15 females; mean age 53.1 ± 15.4 years) were enrolled. All 21 patients with malignant PA masses were FDG-positive (100%), whereas 20 out of 21 patients were FAPI-positive (95.2%). All 12 patients with benign PA masses were both negative in FDG and FAPI PET. The mean maximum standardized uptake value (SUVmax) and target-to-background ratio (TBR) of FAPI and FDG in malignant PA masses were significantly higher than those of benign masses. Although there was no significant difference in SUVmax between FDG and FAPI in malignant PA masses (11.36 vs. 9.18, p = 0.175), the TBR (liver) and TBR (left ventricle) were more favorable for FAPI than for FDG (13.04 vs. 5.17, p < 0.001); (median: 7.75 vs. 2.75, p = 0.007). Immunohistochemical analysis (n = 16) validated that the level of FAP expression corresponded strongly to the uptake of FAPI in PET/CT scans (rs = 0.712, p = 0.002). For clinical management, FAPI PET found more metastatic lesions than FDG PET in 4 patients, with 2 patients upgrading and 1 patient changing treatment decisions. Conclusions: FAPI PET/CT is feasible in the diagnosis of PA masses. Although not superior to FDG PET/CT, FAPI PET/CT showed better target-to-background contrast. Clinical relevance statement: This study found that FAPI PET/CT is not superior to FDG PET/CT in diagnosing PA masses, but FAPI PET/CT displays better target-to-background contrast and more positive lesions, which may help improve disease management. Key Points: Pulmonary malignancies lack specificity in clinical manifestations, laboratory tests, and routine imaging examinations. FAPI PET/CT is not diagnostically better than FDG PET/CT but displays better target-to-background contrast and more positive lesions. Dual-tracer PET/CT ([18F]FAPI-42 and [18F]FDG) imaging improves clinical management of pulmonary artery masses. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the short-term/long-term impact of pulmonary artery intramural hematoma (PA-IMH) in patients with acute Stanford type A aortic dissection (ATAAD) following surgical repair. Materials and methods: Consecutive patients with ATAAD who received surgical repair at Beijing and Yunnan Fuwai Hospital in 2010–2021 were retrospectively reviewed. Patients with hemorrhage extending along the PA were identified as the PA-IMH group. Multivariable logistics regression was used to obtain the odds ratio (OR), and the Kaplan-Meier method was used to estimate the survival rate. Results: Of the 2046 ATAAD patients, 324 (15.8%) patients were identified with PA-IMH, and 1722 (84.2%) were without PA-IMH. PA-IMH had a higher prevalence in patients with older age, female gender, aortic IMH, and type II aortic dissection. PA-IMH patients incurred excess early mortality compared with non-PA-IMH patients (9.3% vs. 5.6%, OR = 1.86, 95%CI 1.19–2.91, p = 0.006). The results were stable in the subgroup analysis, with an increased risk in older (> 70 years) or DeBakey type II ATAAD patients. Notably, an increase in the degree and extent of PA-IMH exacerbated the risk of early mortality. However, after landmark analysis at 30-day postsurgery, no significant difference was noted in the long-term outcomes between PA-IMH and non-PA-IMH groups (p = 0.440). The 5-year survival rates were 87.1% (95%CI: 83.3%, 91.1%) and 90.1% (95%CI: 88.5%, 91.7%), respectively. Conclusions: The presence of PA-IMH in ATAAD patients is common and is independently associated with increased early mortality after surgical repair, especially in those with older age (> 70) or type II dissection. However, such detrimental effects do not persist in the long-term follow-up among patients who survived hospital discharge. Clinical relevance statement: We confirmed that PA-IMH significantly increases early postoperative mortality in patients with acute type A aortic dissection, especially in older patients or DeBakey type II dissection. This should prompt further investigation of the incremental role of PA-IMH in this pathology. Key Points: Acute type A aortic dissection mortality gets worse when pulmonary artery intramural hematoma is present. Pulmonary artery-intramural hematoma increased the risk of early mortality but not affect long-term prognosis. Further research should investigate the effects of pulmonary artery intramural thrombus on aortic dissection. [ABSTRACT FROM AUTHOR]

Purpose: Surgical manipulation of the lungs increases the number of circulating tumor cells and the subsequent risk of metastasis in patients with lung cancer. This study investigated whether or not ligating the tumor-draining pulmonary vein first during lobectomy could improve the prognosis of these patients. Methods: We retrospectively evaluated patients who underwent curative lobectomy for solitary nonsmall-cell lung carcinoma between January 2012 and December 2016. We divided the patients into the vein-first group, in which all associated pulmonary veins were dissected and severed before cutting the pulmonary artery, bronchus, or pulmonary fissure, and the other procedure group. Results: Overall, we included 177 and 413 patients in the vein-first and other procedure groups, respectively. Propensity score matching yielded 67 pairs of patients. The 5-year overall survival (85.6% [95% confidence interval, 77.3–94.8%] vs. 69.4% [58.7–81.9%], P = 0.03%) and recurrence-free survival (73.4% [63.3–85.1%] vs. 53.5% [42.5–67.3%], P = 0.02) were significantly better in the vein-first group than in the other procedure group. The cumulative recurrence rate at 5 years post-surgery was significantly lower in the vein-first group than in the other procedure group (21.7% vs. 38.3%, P = 0.04). Conclusion: Our study suggests that ligating the pulmonary vein first during lobectomy for lung cancer can improve the overall survival, recurrence-free survival, and cumulative recurrence rate. [ABSTRACT FROM AUTHOR]

To characterize the correlation, agreement and concordance of cardiac output (CO) measured with transthoracic ultrasound and the correlation and concordance of aortic blood flow (ABF) minute distance (MD) measured by transesophageal Doppler with CO measured by pulmonary artery thermodilution (PATD) in cats. Experimental study. A group of six healthy male neutered cats, aged 2–8 years and weighing 5.3 ± 0.3 kg. Cats were anesthetized with isoflurane in oxygen. CO was measured by PATD (CO PATD) and transthoracic echocardiography (CO ECHO). ABF MD was measured using an esophageal Doppler flow probe aligned with descending ABF. All measurements were made under three conditions: dexmedetomidine (20 μg kg–1) intravenously; atipamezole (200 μg kg–1) intramuscularly and atropine (20 μg kg–1) intravenously as needed to achieve a minimum heart rate of 140 beats minute–1; and dopamine (20 μg kg–1 minute–1) intravenously in that order. Correlation between CO PATD and CO ECHO , and CO PATD and Doppler MD was evaluated using repeated measures correlation. Agreement between CO PATD and CO ECHO was evaluated using Bland–Altman method. Differences between consecutive pairs of CO measurements were calculated for concordance analysis. Correlation between CO PATD and CO ECHO and between CO PATD and MD was significant (p < 0.001), with correlation coefficients greater than 0.92. A bias of > 27% and upper limits of agreement of 66% were found between CO PATD and CO ECHO. Concordance rate with CO PATD was 76–80% for CO ECHO and 72% for MD. Echocardiographic methods for the measurement of CO showed poor agreement and concordance with PATD. MD showed poor concordance with PATD. As such, these methods cannot be used as an alternative to PATD nor can they appropriately track changes in CO in anesthetized cats. [ABSTRACT FROM AUTHOR]

The rising application of extracorporeal membrane oxygenation (ECMO) has emphasized the need for consistent and standardized terminology, especially concerning peripheral percutaneous cannulation of the pulmonary artery (PPC-PA). The Extracorporeal Life Support Organization (ELSO) Nomenclature Task Force produced the ELSO Maastricht Treaty for extracorporeal life support (ECLS) Nomenclature to address this challenge. However, adherence to nomenclature recommendations has been poor in publications describing PPC-PA. We aim to describe common nomenclature errors and provide a user-guide for abbreviations that can be used by authors, reviewers, and journal staff to ensure properadherence to standardized nomenclature in publications describing PPC-PA. [ABSTRACT FROM AUTHOR]

Background: Assessment of the pattern of the RV outflow tract Doppler provides insights into the hemodynamics of chronic thromboembolic pulmonary hypertension (CTEPH). We studied whether pre-operative assessment of timing of the pulmonary flow systolic notch by Doppler echocardiography is associated with long-term survival after pulmonary endarterectomy (PEA) for CTEPH. Methods: Fifty-nine out of 61 consecutive CETPH patients (mean age 53 ± 14 years, 34% male) whom underwent PEA between June 2002 and June 2005 were studied. Clinical, echocardiographic and hemodynamic variables were assessed pre-operatively and repeat echocardiography was performed 3 months after PEA. Notch ratio (NR) was assessed with pulsed Doppler and calculated as the time from onset of pulmonary flow until notch divided by the time from notch until end of pulmonary flow. Long-term follow-up was obtained between May 2021 and February 2022. Results: Pre-operative mean pulmonary artery pressure (mPAP) was 45 ± 15 mmHg and pulmonary vascular resistance (PVR) was 646 ± 454 dynes.s.cm-5. Echocardiography before PEA showed that 7 patients had no notch, 33 had a NR < 1.0 and 19 had a NR > 1.0. Three months after PEA, echocardiography revealed a significant decrease in sPAP in long-term survivors with a NR < 1.0 and a NR > 1.0, while a significant increase in TAPSE/sPAP was only observed in the NR < 1.0 group. Mean long-term clinical follow-up was 14 ± 6 years. NR was significantly different between survivors and non-survivors (0.73 ± 0.25 vs. 1.1 ± 0.44, p < 0.001) but no significant differences were observed in mPAP or PVR. Long-term survival at 14 years was significantly better in patients with a NR < 1.0 compared to patients with a NR > 1.0 (83% vs. 37%, p = < 0.001). Conclusion: Pre-operative assessment of NR is a predictor of long-term survival in CTEPH patients undergoing PEA, with low mortality risk in patients with NR < 1.0. Long-term survivors with a NR < 1.0 and NR > 1.0 had a significant decrease in sPAP after PEA. However, the TAPSE/sPAP only significantly increased in the NR < 1.0 group. In the NR < 1.0 group, the 6-min walk test increased significantly between pre-operative and at 1-year post-operative follow-up. NR is a simple echocardiographic parameter that can be used in clinical decision-making for PEA. [ABSTRACT FROM AUTHOR]

Background: The impact of continuous flow resulting from contemporary left ventricular assist devices (LVAD) on renal vascular physiology is unknown. Renal resistive index (RRI) reflects arterial compliance, as well as renal vascular resistance, contributed by afferent and efferent arteriolar tone, the renal interstitium as well as renal venous pressures. Methods: Prospective, single center study with renal Doppler evaluation at baseline (pre‐implant) and at 3‐months support. Outcomes assessed include need for post‐operative renal replacement therapy (RRT), worsening renal function (WRF) defined as persistent increase from pre‐implant KDIGO chronic kidney disease stage, right ventricular (RV) failure, and survival to transplantation. Results: Pre‐implant RRI did not predict cardiorenal outcomes including right heart failure, need for renal replacement therapy or worsening renal function. Post‐implant RRI was significantly lower than pre‐implant RRI, with a distinct Doppler waveform characteristic of continuous flow. Post‐implant renal end‐diastolic velocity, but not RRI, correlated strongly with LVAD flow (Spearman rho −0.99, p < 0.001), with trend toward correlation with mean arterial pressure (Spearman's rho 0.63, p = 0.129). There was a negative correlation between post‐implant RRI and mean pulmonary artery pressure (Spearman's rho −0.81, p = 0.049), likely driven by elevated pulmonary capillary wedge pressure (Spearman's rho −0.83, p = 0.058). Conclusion: The hemodynamic contributors to RRI in LVAD supported patients are complex. Higher mean pulmonary artery and pulmonary capillary wedge pressures seen in lower RRI may reflect a smaller difference in systolic and diastolic flow. Future simultaneous Doppler assessment of the LVAD outflow graft and RRI may help understand the hemodynamic interactions contributing to this index. [ABSTRACT FROM AUTHOR]

The use of stereotactic body radiation therapy for tumors in close proximity to the central mediastinal structures has been associated with a high risk of toxicity. This study (NCT03306680) aimed to determine the maximally tolerated dose of stereotactic body radiation therapy for ultracentral non-small cell lung carcinoma, using a time-to-event continual reassessment methodology. Patients with T1-3N0M0 (≤6 cm) non-small cell lung carcinoma were eligible. The maximally tolerated dose was defined as the dose of radiation therapy associated with a ≤30% rate of grade (G) 3 to 5 prespecified treatment-related toxicity occurring within 2 years of treatment. The starting dose level was 60 Gy in 8 daily fractions. The dose-maximum hotspot was limited to 120% and within the planning tumor volume; tumors with endobronchial invasion were excluded. This primary analysis occurred 2 years after completion of accrual. Between March 2018 and April 2021, 30 patients were enrolled at 5 institutions. The median age was 73 years (range, 65-87) and 17 (57%) were female. Planning tumor volume was abutting proximal bronchial tree in 19 (63%), esophagus 5 (17%), pulmonary vein 1 (3.3%), and pulmonary artery 14 (47%). All patients received 60 Gy in 8 fractions. The median follow-up was 37 months (range, 8.9-51). Two patients (6.7%) experienced G3-5 adverse events related to treatment: 1 patient with G3 dyspnea and 1 G5 pneumonia. The latter had computed tomography findings consistent with a background of interstitial lung disease. Three-year overall survival was 72.5% (95% CI, 52.3%-85.3%), progression-free survival 66.1% (95% CI, 46.1%-80.2%), local control 89.6% (95% CI, 71.2%-96.5%), regional control 96.4% (95% CI, 77.2%-99.5%), and distant control 85.9% (95% CI, 66.7%-94.5%). Quality-of-life scores declined numerically over time, but the decreases were not clinically or statistically significant. : Sixty Gy in 8 fractions, planned and delivered with only a moderate hotspot, has a favorable adverse event rate within the prespecified acceptability criteria and results in excellent control for ultracentral tumors. [ABSTRACT FROM AUTHOR]

Clinical data: A nine-month-old female infant diagnosed with transposition of the great arteries with symptoms of heart failure associated with cyanosis and difficulty in gaining weight was referred to our center with late diagnosis (at nine months of age). Chest radiography: Cardiomegaly; attenuated peripheral vascular markings. Electrocardiography: Sinus rhythm with biventricular overload and aberrantly conducted supraventricular extra systoles. Echocardiography: Wide atrial septal defect, ventricular axis torsion with concordant atrioventricular connection and discordant ventriculoarterial connection. Computed tomography angiography: Concordant atrioventricular connection, right ventricle positioned superiorly and left ventricle positioned inferiorly; discordant ventriculoarterial connection with right ventricle connected to the aorta and left ventricle connected to pulmonary artery. Diagnosis: Crisscross heart is a rare congenital heart defect, accounting for 0.1% of congenital heart diseases. It consists of the 90° rotation of ventricles' axis in relation to their normal position; therefore, ventricles are positioned in the superior-inferior direction rather than anterior-posterior. Most cases have associated cardiac anomalies, and in this case, it is associated with transposition of the great arteries. The complexity and rarity of its occurrence make diagnosis and surgical treatment challenging. Operation: Modified Senning procedure using the pericardial sac in the construction of a tunnel from pulmonary veins to the right atrium. Cardiopulmonary bypass time of 147 minutes with nine minutes of total circulatory arrest. [ABSTRACT FROM AUTHOR]

The study aimed to standardize the cryodehydration technique for bovine fetal hearts, focusing on optimizing protocols for each developmental stage to preserve morphological characteristics. We analyzed 29 bovine fetal hearts categorized into early, middle, and late developmental stages. These hearts underwent cryodehydration until a 60%–70% reduction in original fluid volume was achieved. Biometric data were recorded and statistically analyzed using Pearson correlation tests for age versus weight and age versus number of cryodehydration sessions. Morphometric comparisons before and after cryodehydration were performed using paired t‐tests. In Group I, hearts exhibited well‐defined structures, including the atrium cordis, ventriculus cordis, auricula atrii, aorta, truncus pulmonalis, and ramus coronaries arteria, which were preserved in Groups II and III. Additionally, in Group I the heart had a conical or flat apex cordis, whereas those in Groups II and III had a more pronounced apex. The average number of cryodehydration sessions required was 9.38 (±1.2) days for Group I, 12.37 (±1.4) days for Group II, and 15 days for Group III. A positive correlation was found between age and sample weight, indicating that more developed hearts were heavier. Similarly, there was a positive correlation between gestational age and the number of cryodehydration sessions, suggesting that more advanced stages required more cryodehydration sessions. Paired t‐tests demonstrated high statistical significance in the morphometric parameters before and after cryodehydration, indicating a loss of mass during dehydration. However, there was no alteration in the macroscopic structure of the hearts, which remained morphologically preserved. In conclusion, cryodehydration shows promise for preserving and analyzing the external morphological characteristics of bovine fetal cardiac development. It also provides lightweight, odorless, and easy‐to‐handle specimens ideal for detailed morphological studies and offers a unique perspective for investigating cardiac morphology in biological research contexts. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the effect of the contrast-enhancement-boost (CE-boost) postprocessing technique on improving the image quality of obese patients in computed tomography pulmonary angiography (CTPA) compared to hybrid iterative reconstruction (HIR) and model-based iterative reconstruction (MBIR) algorithms. Methods: This prospective study was conducted on 100 patients who underwent CTPA for suspected pulmonary embolism. Non-obese patients with a body mass index (BMI) under 25 were designated as group 1, while obese patients (group 2) had a BMI exceeding 25. The CE-boost images were generated by subtracting non-contrast HIR images from contrast-enhanced HIR images to improve the visibility of pulmonary arteries further. The CT value, image noise, signal-to-noise ratio (SNR), and contrast-to-noise ratio (CNR) were quantitatively assessed. Two chest radiologists independently reviewed the CT images (5, best; 1, worst) across three subjective characteristics including diagnostic confidence, subjective image noise, and vascular contrast. The Friedman test and Dunn-Bonferroni correction were used for statistical analysis. Results: The CE-boost had significantly higher CT values than HIR and MBIR in both groups (all p < 0.001). The MBIR yielded the lowest image noise compared with HIR and CE-boost (all p < 0.001). The SNR and CNR of main pulmonary artery (MPA) were significantly higher in CE-boost than in MBIR (all p < 0.05), with HIR showing the lowest values (all p < 0.001). Group 2 MBIR received significantly better subjective image noise scores, while the diagnostic confidence and vascular contrast scored highest with the group 2 CE-boost (all p < 0.05). Conclusion: Compared to the HIR algorithm, both the CE-boost technique and the MBIR algorithm can improve the image quality of CTPA in obese patients. CE-boost had the greatest potential in increasing the visualization of pulmonary artery and its branches. [ABSTRACT FROM AUTHOR]

Background/Objectives: To propose criteria for retreating previously embolized PAVMs and determining the effectiveness of the criteria to prevent paradoxical embolization. Methods: A retrospective review of patients with PAVMs treated at a single HHT center of excellence between 1 January 2013, and 10 September 2023, was performed. Patients with PAVM recurrence were either retreated or observed based on the following criteria for PAVM reintervention: 1. Embolic device(s) not creating a sufficiently dense matrix, such that a channel through them may be >/ 2 mm; 2. Accessory feeding artery or pulmonary collateral >/ 2 mm; 3. Hemoptysis in a patient with no other explanation. Results: A total of 438 PAVMs were treated in 151 patients, including 106 patients with definite, 14 possible, and 31 doubtful HHT. Post-embolization PAVM recurrence occurred in 36 patients (36/151, 23.8%), including 15 patients (15/151, 9.9%) with 22 PAVMs (22/438, 5.0%) meeting criteria for reintervention. A total of 21 patients (21/151, 13.9%) with recurrence did not meet reintervention criteria and were therefore observed. Pre-treatment paradoxical embolization occurred in 36 patients (36/151) for a lifetime prevalence rate of 23.7%. Post-treatment paradoxical embolization did not occur in any patients following PAVM embolization (0/151). There was one case of iatrogenic paradoxical embolization in a patient being treated for systemic collateral reperfusion and hemoptysis. However, this was not included given that it was not a spontaneous event. Conclusions: Utilizing modern embolization techniques and devices, the proposed reintervention criteria, and screening intervals, paradoxical embolizations can be effectively prevented in patients with PAVMs. [ABSTRACT FROM AUTHOR]

Background: Pulmonary arterial hypertension is a life-threatening progressive disorder characterised by high blood pressure (hypertension) in the arteries of the lungs (pulmonary artery). Although treatable, there is no known cure for this rare disorder, and its exact cause is unknown. Mutations in the bone morphogenetic protein receptor type-2 (BMPR2) are the most common genetic cause of familial pulmonary arterial hypertension. This study represents the first-ever trial of treatments aimed at directly rescuing the BMPR2 pathway, repurposing two drugs that have shown promise at restoring levels of BMPR2 signalling: hydroxychloroquine and phenylbutyrate. Methods: This three-armed phase II precision medicine study will investigate BMPR2 target engagement and explore the efficacy of two repurposed therapies in pulmonary arterial hypertension patients with BMPR2 mutations. Patients will be stratified based on two BMPR2 mutation classes: missense and haploinsufficient mutations. Eligible subjects will be randomised to one of the three arms (two active therapy arms and a placebo arm, all plus standard of care) following a Bayesian response-adaptive design implemented independently in each stratum and updated in response to a novel panel of primary biomarkers designed to assess biological modification of the disease. Discussion: The results of this trial will provide the first randomised evidence of the efficacy of these therapies to rescue BMPR2 function and will efficiently explore the potential for a differential response of these therapies per mutation class to address causes rather than consequences of this rare disease. Trial registration: The study has been registered with ISRCTN (ISRCTN10304915, 22/09/2023). [ABSTRACT FROM AUTHOR]

Background: Fibrosing mediastinitis (FM) is a rare yet fatal condition, caused by different triggers and frequently culminating in the obstruction of the pulmonary vasculature and airways, often leading to pulmonary hypertension and right heart failure. Percutaneous transluminal pulmonary venoplasty (PTPV) is an emerging treatment for pulmonary vein stenosis (PVS) caused by FM. Our previous study showed as high as 24% of in-stent restenosis (ISR) in FM. However, the predictors of ISR are elusive. Objectives: We sought to identify the predictors of ISR in patients with PVS caused by extraluminal compression due to FM. Methods: We retrospectively enrolled patients with PVS-FM who underwent PTPV between July 1, 2018, and December 31, 2022. According to ISR status, patients were divided into two groups: the ISR group and the non-ISR group. Baseline characteristics (demographics and lesions) and procedure-related information were abstracted from patient records and analyzed. Univariate and multivariate analyses were performed to determine the predictors of ISR. Results: A total of 142 stents were implanted in 134 PVs of 65 patients with PVS-FM. Over a median follow-up of 6.6 (3.4–15.7) months, 61 of 134 PVs suffered from ISR. Multivariate analysis demonstrated a significantly lower risk of ISR in PVs with a larger reference vessel diameter (RVD) (odds ratio (OR): 0.79; 95% confidence interval [CI]: 0.64 to 0.98; P = 0.032), and stenosis of the corresponding pulmonary artery (Cor-PA) independently increased the risk of restenosis (OR: 3.41; 95% CI: 1.31 to 8.86; P = 0.012). The cumulative ISR was 6.3%, 21.4%, and 39.2% at the 3-, 6-, and 12-month follow-up, respectively. Conclusion: ISR is very high in PVS-FM, which is independently associated with RVD and Cor-PA stenosis. Trail Registration: Chinese Clinical Trials Register; No.: ChiCTR2000033153. URL: http://www.chictr.org.cn. [ABSTRACT FROM AUTHOR]

The purpose of this study was to evaluate the value of MGI and multi-parameter in the assessment of different pulmonary circulation blood volumes in congenital heart disease.This study included 350 fetuses categorized into two groups: Normal group consisted of 258 fetuses with no discernible abnormalities through echocardiography as control Group A; Abnormal group with abnormal echocardiogram, including Group B of 71 fetuses with decreased pulmonary blood flow or pulmonary atresia and Group C of 21 fetuses with reduced or detached aortic blood flow.The MGI and Z-scores were measured and compared among these groups. Significant variations were noted in the aortic outflow Z-scores (AO-Zs) (p<0.01), pulmonary artery (PA) (p<0.01), PA Z-scores (PA-Zs) (p<0.01), PA/AO (p<0.01), right PA (p<0.01), and MGI (p<0.01) among the three groups (all p<0.05). Among fetuses with decreased pulmonary blood flow or pulmonary atresia, PA, PA-Zs, and MGI in fetuses with reverse DA flow perfusion were lower than those in the DA forward perfusion group.Fetal echocardiography, incorporating the MGI and multi-parameter, not only allows for the evaluation of pulmonary blood flow and pulmonary vascular development of the fetus but also enables the observation of changes in pulmonary blood flow and MGI development across different gestational weeks. [ABSTRACT FROM AUTHOR]

Acquired pulmonary artery branch stenosis without main pulmonary artery involvement due to external compression by neoplasia has been described in human and veterinary medicine. Over time, this can result in right ventricular hypertension and right-sided heart failure. Endovascular stenting offers quick relief from signs, while the underlying cause is addressed. Here, we present a dog with severe right pulmonary artery compression caused by a chemodectoma, which was treated with two, overlapping, self-expanding vascular stents and chemotherapy. The patient experienced immediate symptomatic relief, progressive stent expansion over time, and has been free of clinical symptoms for 5 months post implantation. [ABSTRACT FROM AUTHOR]

Background: Pulmonary arteriovenous malformation (PAVM) is abnormal arteriovenous shunts between pulmonary artery (PA) and pulmonary vein, and rarely has congenital direct communications with systemic arteries. Case Presentation: A 33-year-old male presented to our hospital with intermittent bloody sputum with no evidence of pulmonary infection, trauma or surgery. Chest computed tomography angiography (CTA) indicated the congenital inferior phrenic artery (IPA)-to-PAVM surrounded by diffuse alveolar hemorrhage located in the lower lobe of right lung. Both the afferent PA and IPA were successfully embolized with coils. Recurrent hemoptysis did not occur during one-year follow up. Conclusions: The congenital communication between IPA and PAVM is rare, and the abnormal direct shunt would induce hemodynamically unstable condition within PAVM. Endovascular embolization of the afferent PA and IPA is a safe and effective method for this abnormal congenital shunt in lung. [ABSTRACT FROM AUTHOR]

Background: Pulmonary pseudoaneurysm (PAP) is a fatal complication of severe acute respiratory syndrome caused by coronavirus 2 infections in the lungs. Other causative agent such as fungal and tubercular also responsible for this entity. Early detection and treatment can save a patient's life. Case presentation: Here we describe a case of a left pulmonary artery pseudo aneurysm in a 54-year-old patient with COVID-19 pneumonia who presented with massive hemoptysis and which was treated with endovascular embolization. Conclusion: It is caused by vasculopathy due to immunosuppression /immune dysregulation in COVID-19. Minimally invasive endovascular embolization of pseudoaneurysm carries less mortality and morbidity. [ABSTRACT FROM AUTHOR]

Pulmonary embolisms take two forms, depending on whether the cause is thrombotic or non-thrombotic. Although emboli with non-thrombotic causes are rare, they should not be ignored during diagnosis. In such cases, a detailed anamnesis should be taken, existing risk factors should be inquired, and additional tests should be made before making a firm diagnosis. In our case, a hydatid cyst embolism that occluded a large part of the pulmonary artery had been identified as a medium–high-risk pulmonary thromboembolism during hospitalization in an external center and thrombolytic treatment had been started. As the patient’s symptoms were identified as unresolved during an outpatient clinic check-up, a hydatid cyst was diagnosed based on the results of a transthoracic fine needle aspiration biopsy and treatment was started. [ABSTRACT FROM AUTHOR]

Conduit pulmonary arterial stiffening and the resultant increase in pulmonary vascular impedance have emerged as an important underlying driver of pulmonary arterial hypertension (PAH). Given that matrix deposition is central to vascular remodeling, we evaluated the role of the collagen cross-linking enzyme lysyl oxidase like 2 (LOXL2) in this study. Human pulmonary artery smooth muscle cells (PASMCs) subjected to hypoxia showed increased LOXL2 secretion. LOXL2 activity and expression were markedly higher in primary PASMCs isolated from the pulmonary arteries of the rat Sugen 5416 + hypoxia (SuHx) model of severe pulmonary hypertension (PH). Similarly, LOXL2 protein and mRNA levels were increased in the pulmonary arteries (PA) and lungs of rats with PH (SuHx and monocrotaline (MCT) models). Pulmonary arteries (PAs) isolated from the rats with PH exhibited hypercontractility to phenylephrine and attenuated vasorelaxation elicited by acetylcholine, indicating severe endothelial dysfunction. Tensile testing revealed a significant increase in PA stiffness in PH. Treatment with PAT-1251, a novel small-molecule LOXL2 inhibitor, improved active and passive properties of the PA ex vivo. There was an improvement in right heart function as measured by right ventricular pressure volume loops in vivo with PAT-1251. Importantly, PAT-1251 treatment ameliorated PH, resulting in improved pulmonary artery pressures, right ventricular remodeling, and survival. Hypoxia-induced LOXL2 activation is a causal mechanism in pulmonary artery stiffening in PH and pulmonary artery mechanical and functional decline. LOXL2 inhibition with PAT-1251 could be a promising approach to improve pulmonary artery pressures, right ventricular elastance, cardiac relaxation, and survival in PAH. NEW & NOTEWORTHY: Pulmonary arterial stiffening contributes to the progression of PAH and the deterioration of right heart function. This study shows that LOXL2 is upregulated in rat models of PH. LOXL2 inhibition halts pulmonary vascular remodeling and improves PA contractility, endothelial function, and PA pressure, resulting in prolonged survival. Thus, LOXL2 is an important mediator of PA remodeling and stiffening in PH and a promising target to improve PA pressures and survival in PH. [ABSTRACT FROM AUTHOR]

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital condition. When UAPA occurs without associated congenital heart disease, it is referred to as isolated unilateral absence of the pulmonary artery (IUAPA). IUAPA is frequently not diagnosed until adulthood. A 61‐year‐old female patient presented with right chest pain and a worsening interstitial shadow over 9 years. Contrast‐enhanced computed tomography revealed the absence of blood flow from the pulmonary artery in the right lung, leading to the diagnosis of IUAPA. In this case, the diagnosis of UAPA was established after approximately 40 years. This case underscores the importance of identifying vascular abnormalities to differentiate this condition in patients presenting with nonspecific symptoms and interstitial shadows. [ABSTRACT FROM AUTHOR]

Oesophagomediastinal fistula is uncommon. Oesophageal fistulas, may manifest as recurrent pneumonias. While pulmonary infections can lead to pulmonary artery pseudoaneurysms (PAPs), particularly in fungal infections. PAPs pose a rupture risk, potentially causing life‐threatening hemoptysis. We report a unique case of a 45‐year‐old male who presented with sudden cough, dyspnea, and hemoptysis. Bronchoscopy triggered massive hemoptysis, necessitating emergency embolization. Persistent hemoptysis prompted further imaging, revealing an aneurysmal dilation located next to the spine and infectious lesions, suggesting an oesophagomediastinal fistula. After initiating therapy with Amphotericin B Cholesterol Sulfate Complex and fistula closure, the patient's hemoptysis resolved, with imaging resolution of the PAP. Long‐term Voriconazole therapy ensured continued improvement. This case highlights the rarity and severity of such fistulas may be associated with fungal infections and PAPs, emphasizing the importance of prompt recognition, aggressive treatment for favourable outcomes. [ABSTRACT FROM AUTHOR]

Tricuspid regurgitation (TR) is an increasingly prevalent condition, especially in older populations, and presents significant challenges due to its association with right heart failure, hospital admissions, and high mortality rates. The management of TR has evolved, with new percutaneous valve repair and replacement techniques emerging alongside traditional surgical approaches. However, accurately assessing right ventricular (RV) function–a key prognostic factor in TR–remains difficult due to the RV's unique anatomy and sensitivity to loading conditions. Current echocardiographic methods, such as Tricuspid Annular Plane Systolic Excursion (TAPSE), S' wave analysis, and RV fractional area change (FAC), offer valuable insights but have limitations, particularly regarding load dependence and incomplete assessment of RV function. Advances in 3D echocardiography and myocardial strain imaging provide more comprehensive evaluations, yet challenges persist in integrating these measures in routine clinical practice. The review highlights the importance of a multimodal approach to RV assessment in TR patients, considering both the right atrium and pulmonary artery interactions, and explores potential future tools such as myocardial work and dynamic testing to improve prognostic accuracy and patient outcomes. [ABSTRACT FROM AUTHOR]

Simple Summary: The prevalence of indeterminate pulmonary nodule (IPN) findings in early lung cancer screening settings highlights the need for novel imaging biomarkers to assist clinicians in making informed therapeutic decisions. The surrounding vasculature of pulmonary nodules, termed "macro-vasculature", has been identified as a potential biomarker for evaluating nodule malignancy. However, the relationship between the macro-vasculature's arteries and veins surrounding an IPN and its malignancy remains unclear. This study aimed to investigate this association. Our findings indicate that arterial characteristics significantly outweigh venous characteristics in distinguishing malignant from benign nodules. Additionally, incorporating arterial information surrounding a nodule enhances the performance in differentiating malignant from benign nodules. Clarifying the relationship between macro-vasculature arteries and nodule malignancy may facilitate clinical follow-up procedures for screen-detected pulmonary nodules. Objective: This study aims to investigate the association between the arteries and veins surrounding a pulmonary nodule and its malignancy. Methods: A dataset of 146 subjects from a LDCT lung cancer screening program was used in this study. AI algorithms were used to automatically segment and quantify nodules and their surrounding macro-vasculature. The macro-vasculature was differentiated into arteries and veins. Vessel branch count, volume, and tortuosity were quantified for arteries and veins at different distances from the nodule surface. Univariate and multivariate logistic regression (LR) analyses were performed, with a special emphasis on the nodules with diameters ranging from 8 to 20 mm. ROC-AUC was used to assess the performance based on the k-fold cross-validation method. Average feature importance was evaluated in several machine learning models. Results: The LR models using macro-vasculature features achieved an AUC of 0.78 (95% CI: 0.71–0.86) for all nodules and an AUC of 0.67 (95% CI: 0.54–0.80) for nodules between 8–20 mm. Models including macro-vasculature features, demographics, and CT-derived nodule features yielded an AUC of 0.91 (95% CI: 0.87–0.96) for all nodules and an AUC of 0.82 (95% CI: 0.71–0.92) for nodules between 8–20 mm. In terms of feature importance, arteries within 5.0 mm from the nodule surface were the highest-ranked among macro-vasculature features and retained their significance even with the inclusion of demographics and CT-derived nodule features. Conclusions: Arteries within 5.0 mm from the nodule surface emerged as a potential biomarker for effectively discriminating between malignant and benign nodules. [ABSTRACT FROM AUTHOR]

Hemoptysis in the pediatric population, while infrequent, poses significant challenges for both the family and healthcare practitioners. The severity of hemoptysis dictates management decisions. Most cases being mild and self-limiting are treated conservatively. However, "life-threatening hemoptysis" may occur, and is defined as any degree of blood loss that endangers the airway and is arbitrarily considered to be > 8 ml/kg in 24 h in children. It requires prompt airway management and resuscitation followed by a tailored approach consisting of bronchoscopy, computed tomography (CT), interventional radiology, and/or surgery depending on the patient 's clinical status and cardiopulmonary comorbidities. Bronchial arteries are hypertrophied in myriad conditions and account for 90–95% cases of hemoptysis due to their systemic pressure levels; the rest being contributed by pulmonary artery pathologies. Despite similar pathogenic mechanisms, the etiologies of pediatric hemoptysis differ from those in adults, with acute lower respiratory tract infections being the predominant cause. Imaging plays a crucial role in identifying the source and cause of hemorrhage. Multidetector computed tomography (MDCT) has emerged as a prime modality in the diagnostic evaluation of hemoptysis and provides a roadmap for potential interventional procedures. This article discusses the etiopathogenesis of hemoptysis along with a brief mention of the diagnostic modalities. It provides a structured reporting format and uses it to illustrate the imaging features in hemoptysis, with emphasis on CT angiography. The key findings in the lung parenchyma, airways, bronchial and non-bronchial systemic collaterals, and pulmonary arteries are elaborated upon. It further addresses the nuances of interventional management, particularly emphasizing the applications of bronchial artery embolization and pulmonary artery embolization in the pediatric population. The article also underscores the potential complications and factors influencing recurrence rates. [ABSTRACT FROM AUTHOR]

Despite exercise intolerance being predictive of outcomes in pulmonary arterial hypertension (PAH), its underlying cardiac mechanisms are not well described. The aim of the study was to explore the biventricular response to exercise and its associations with cardiorespiratory fitness in children with PAH. Participants underwent incremental cardiopulmonary exercise testing and simultaneous exercise echocardiography on a recumbent cycle ergometer. Linear mixed models were used to assess cardiac function variance and associations between cardiac and metabolic parameters during exercise. Eleven participants were included with a mean age of 13.4 ± 2.9 yr old. Right ventricle (RV) systolic pressure (RVsp) increased from a mean of 59 ± 25 mmHg at rest to 130 ± 40 mmHg at peak exercise (P < 0.001), whereas RV fractional area change (RV-FAC) and RV-free wall longitudinal strain (RVFW-Sl) worsened (35.2 vs. 27%, P = 0.09 and −16.6 vs. −14.6%, P = 0.1, respectively). At low- and moderate-intensity exercise, RVsp was positively associated with stroke volume and O2 pulse (P < 0.1). At high-intensity exercise, RV-FAC, RVFW-Sl, and left ventricular longitudinal strain were positively associated with oxygen uptake and O2 pulse (P < 0.1), whereas stroke volume decreased toward peak (P = 0.04). In children with PAH, the increase of pulmonary pressure alone does not limit peak exercise, but rather the concomitant reduced RV functional reserve, resulting in RV to pulmonary artery (RV-PA) uncoupling, worsening of interventricular interaction and LV dysfunction. A better mechanistic understanding of PAH exercise physiopathology can inform stress testing and cardiac rehabilitation in this population. NEW & NOTEWORTHY: In children with pulmonary arterial hypertension, there is a marked increase in pulmonary artery pressure during physical activity, but this is not the underlying mechanism that limits exercise. Instead, right ventricle-to-pulmonary artery uncoupling occurs at the transition from moderate to high-intensity exercise and correlates with lower peak oxygen uptake. This highlights the more complex underlying pathological responses and the need for multiparametric assessment of cardiac function reserve in these patients when feasible. [ABSTRACT FROM AUTHOR]

Purpose Preoperative evaluation of pulmonary vascular and tracheal routes and variations is of great importance to the surgeon. Three-dimensional computed tomography bronchography and angiography (3D-CTBA) has evolved in recent years with the optimization of 3D reconstruction techniques and artificial intelligence. We aim to apply CT angiography and Exoview 3D reconstruction technology to assess patients' pulmonary arterial tree and its anatomical variants and to try to summarize a set of anatomical typing of the pulmonary arterial tree that is relatively easy and conducive to promoting teaching based on surgical habits of lobectomy. Methods A total of 358 patients hospitalized in the Department of Thoracic Surgery of the First Affiliated Hospital of Soochow University between July 2020 and August 2021 were included in this study. We carefully analyzed the site of emanation, alignment, and number of branches of the pulmonary artery according to a uniform classification method in conjunction with the two-dimensional CT images and transformed them into 3D reconstruction models. Results Different types of pulmonary artery were observed in 358 cases. We evaluated the complete pulmonary artery tree and counted the number and frequency of major arteries of the pulmonary based on the surgical habits of anatomical lobectomy. Conclusion The 3D-CTBA technique enables us to adequately assess the anatomy of the pulmonary arteries. Moreover, we provide a practical classification scheme of pulmonary arterial anatomical patterns based on lobectomy and 3D-CTBA. Our data can be used by clinicians in the teaching of pulmonary artery anatomy and the preoperative preparation for anatomical lobectomy. [ABSTRACT FROM AUTHOR]

Background: Currently available risk stratification models for acute pulmonary embolism (PE) include hemodynamic status, cardiac biomarkers, right ventricle (RV) dysfunction on imaging, and clinical scores. Focusing on the length--tension relationship of the ventricle might have a superior predictive capability over RV dysfunction in terms of mortality and classification of patients with acute PE. In this study, our hypothesis suggests that the tricuspid annular plane systolic excursion (TAPSE)/systolic pulmonary artery pressure (sPAP) ratio has superior predictive capability for in-hospital mortality in patients with acute PE compared to TAPSE or sPAP as distinct measures. Methods: This single-center study comprised retrospectively evaluated 703 patients referred to our tertiary cardiovascular center with acute PE. We divided patients into quartiles based on the TAPSE/sPAP ratio. Different models were developed to quantify the predictive relationship between in-hospital death and echocardiographic measurements. A base model was created with variables including risk status and RV/LV ratio >1. Then, to evaluate the predictive contribution of each measurement; TAPSE/sPAP, TAPSE, and sPAP were sequentially added to the base model. After that, the performance of each model was evaluated. Results: Predictive and discriminative power was the highest in model containing TAPSE/ sPAP. There was still a significant inverse association between TAPSE/sPAP and the risk of in-hospital death even after adjusting for risk status and RV/LV ratio >1. Receiver operating characteristic curve analysis for TAPSE/sPAP revealed the best cut-off value as 0.34. Conclusion: The outcomes of our study reveal that the ratio of TAPSE/sPAP serves as a more potent predictor of mortality than either of the 2 measurements taken separately. The interpretation and utilization of the TAPSE/sPAP cut-off value in acute PE can assist in identifying patients at risk of deterioration and guide the consideration of more intensive treatment options across all risk groups. [ABSTRACT FROM AUTHOR]

OBJECTIVE To investigate whether liver compression (LC) could increase stroke volume (SV) by more than 15% in healthy, anesthetized dogs with hypovolemia and suggest LC as a novel method to evaluate fluid responsiveness. ANIMALS 6 healthy Beagles. METHODS This prospective, nonrandomized experimental study was conducted from November 2023 to February 2024. The dogs were anesthetized with isoflurane and mechanically ventilated under neuromuscular blockade. After instrumentation, the dogs underwent the following 4 experimental stages in a sequential, nonrandomized manner: stage 1, baseline; stage 2, 30% withdrawal of circulating blood volume; stage 3, 50% infusion of the collected blood; and stage 4, the remaining 50% infusion of the collected blood. At each stage, SV via pulmonary artery thermodilution and hemodynamic variables were measured before, during, and after the LC. RESULTS In stage 2, LC significantly increased mean SV by 30%, from 6.9 to 9 mL/beat. Simultaneously, LC significantly increased mean arterial pressure by 11 mm Hg and mean central venous pressure by 2 mm Hg, while pulse pressure variation significantly decreased from 28% to 22%. In stages 1, 3, and 4, LC did not significantly change mean SV, mean arterial pressure, and pulse pressure variation; however, mean central venous pressure significantly increased during stage 3. CLINICAL RELEVANCE This study demonstrates that LC at 22 mm Hg for 1 minute could increase SV more than 15% in anesthetized, hypovolemic dogs and LC could be used as a novel method to evaluate fluid responsiveness. [ABSTRACT FROM AUTHOR]

Background: Patients can develop de novo malignancies following orthotopic heart transplantation. However, vascular tumors are not commonly described in this population. Case presentation: We present a 69-year-old female with a history of orthotopic heart transplantation for chemotherapy-induced cardiomyopathy who developed an incidental pulmonary artery mass six years after her transplantation. Given concerns for malignancy, the patient underwent an operative excisional biopsy through a left anterior mini-thoracotomy with femoral artery and vein cannulation for cardiopulmonary bypass. The mass was determined to be a non-malignant vascular overgrowth with PIK3CA mutation. Conclusion: We present the case of an unusual pulmonary artery mass with PIK3CA mutation found in a post heart transplant patient. We were able to spare her the morbidity of a redo-sternotomy by excising the mass via a minimally invasive left anterior thoracotomy approach. [ABSTRACT FROM AUTHOR]

Introduction: Pyroptosis, inflammatory necrosis of cells, is a programmed cell death involved in the pathological process of diseases. Endoplasmic reticulum stress (ERS), as a protective stress response of cell, decreases the unfold protein concentration to inhibit the unfold protein agglutination. Whereas the relationship between endoplasmic reticulum stress and pyroptosis in pulmonary hypertension (PH) remain unknown. Previous evident indicated that circular RNA (circRNA) can participate in several biological process, including cell pyroptosis. However, the mechanism of circRNA regulate pyroptosis of pulmonary artery smooth muscle cells through endoplasmic reticulum stress still unclear. Here, we proved that circSSR1 was down-regulate expression during hypoxia in pulmonary artery smooth muscle cells, and over-expression of circSSR1 inhibit pyroptosis both in vitro and in vivo under hypoxic. Our experiments have indicated that circSSR1 could promote host gene SSR1 translation via m6A to activate ERS leading to pulmonary artery smooth muscle cell pyroptosis. In addition, our results showed that G3BP1 as upstream regulator mediate the expression of circSSR1 under hypoxia. These results highlight a new regulatory mechanism for pyroptosis and provide a potential therapy target for pulmonary hypertension. Methods: RNA-FISH and qRT-PCR were showed the location of circSSR1 and expression change. RNA pull-down and RIP verify the circSSR1 combine with YTHDF1. Western blotting, PI staining and LDH release were used to explore the role of circSSR1 in PASMCs pyroptosis. Results: CircSSR1 was markedly downregulated in hypoxic PASMCs. Knockdown CircSSR1 inhibited hypoxia induced PASMCs pyroptosis in vivo and in vitro. Mechanistically, circSSR1 combine with YTHDF1 to promote SSR1 protein translation rely on m6A, activating pyroptosis via endoplasmic reticulum stress. Furthermore, G3BP1 induce circSSR1 degradation under hypoxic. Conclusion: Our findings clarify the role of circSSR1 up-regulated parental protein SSR1 expression mediate endoplasmic reticulum stress leading to pyroptosis in PASMCs, ultimately promoting the development of pulmonary hypertension. [ABSTRACT FROM AUTHOR]

Pulmonary artery stenosis (PAS) often presents in children with congenital heart disease, altering blood flow and pressure during critical periods of growth and development. Variability in stenosis onset, duration, and severity result in variable growth and remodeling of the pulmonary vasculature. Computational fluid dynamics (CFD) models enable investigation into the hemodynamic impact and altered mechanics associated with PAS. In this study, a one-dimensional (1D) fluid dynamics model was used to simulate hemodynamics throughout the pulmonary arteries of individual animals. The geometry of the large pulmonary arteries was prescribed by animal-specific imaging, whereas the distal vasculature was simulated by a three-element Windkessel model at each terminal vessel outlet. Remodeling of the pulmonary vasculature, which cannot be measured in vivo, was estimated via model-fitted parameters. The large artery stiffness was significantly higher on the left side of the vasculature in the left pulmonary artery (LPA) stenosis group, but neither side differed from the sham group. The sham group exhibited a balanced distribution of total distal vascular resistance, whereas the left side was generally larger in the LPA stenosis group, with no significant differences between groups. In contrast, the peripheral compliance on the right side of the LPA stenosis group was significantly greater than the corresponding side of the sham group. Further analysis indicated the underperfused distal vasculature likely moderately decreased in radius with little change in stiffness given the increase in thickness observed with histology. Ultimately, our model enables greater understanding of pulmonary arterial adaptation due to LPA stenosis and has potential for use as a tool to noninvasively estimate remodeling of the pulmonary vasculature. [ABSTRACT FROM AUTHOR]

The 2022 European Society of Cardiology/European Respiratory Society guidelines define pulmonary hypertension (PH) as a resting mean pulmonary artery pressure (mPAP) > 20 mm Hg at right heart catheterization (RHC). Previously, patients with an mPAP between 21 and 24 mm Hg were classified in a "gray zone" of unclear clinical significance. What is the diagnostic performance of the main parameters used for PH screening in detecting patients with systemic sclerosis (SSc) with an mPAP of 21 to 24 mm Hg at RHC? Patients with SSc from the European Scleroderma Trials and Research (EUSTAR) database with available tricuspid annular plane systolic excursion (TAPSE), systolic PAP (sPAP), and mPAP data were included. Patients with mPAP 21 to 24 mm Hg and patients with mPAP ≤ 20 mm Hg were considered for the analysis. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were calculated. TAPSE/sPAP was lower in the group of patients with SSc with mPAP 21 to 24 mm Hg than in the non-PH group (0.58 [0.46-0.72] vs 0.69 [0.57-0.81] mm/mm Hg, respectively; P <.01). No difference was found in other parameters between the two groups. Diffusing capacity of the lungs for carbon monoxide < 80% of the predicted value had the highest sensitivity (88.9%) and NPV (80%), but the lowest specificity (18.2%) and PPV (30.8%) in detecting patients with SSc with mPAP 21 to 24 mm Hg. TAPSE/sPAP < 0.55 mm/mm Hg had the highest specificity (78.9%), PPV (50%), and accuracy (68.1%); its NPV was 75.4%, and its sensitivity was 45.1%. In this study, diffusing capacity of the lungs for carbon monoxide < 80% of the predicted value was the parameter with the highest sensitivity and NPV in detecting patients with SSc with mPAP 21 to 24 mm Hg. TAPSE/sPAP < 0.55 mm/mm Hg had the highest specificity, PPV, and accuracy and, therefore, can be a useful additional parameter to decrease the number of unnecessary RHCs. [Display omitted] [ABSTRACT FROM AUTHOR]

Various surgical approaches address complex heart disease with arch anomalies. Bilateral pulmonary artery banding (bPAB) is a strategy for critically ill patients with complex arch anomalies. Some reports argued the potential effect of bPAB on the growth of the left ventricular outflow tract (LVOT) during inter-stage after bPAB. This study aimed to analyze the LVOT growth for biventricular repair candidates with arch anomaly and systemic ventricular outflow tract (SVOT) for univentricular repair candidates with arch anomaly. This retrospective study analyzed 17 patients undergoing initial bPAB followed by arch repair. The Z-scores of LVOT and SVOT were compared between pre-bPAB and pre-arch repair. Patient characteristics, transthoracic echocardiogram data, and PAB circumferences were reviewed. The diameter of the minimum LVOT for biventricular repair (BVR) candidates, the pulmonary valve (neo-aortic valve, neo-AoV) and the pulmonary trunk (the neo-ascending aorta, neo-AAo) for univentricular repair (UVR) candidates, and the degree of aortic or neo-aortic insufficiency in each candidate was statistically analyzed. 17 patients were divided into the UVR candidates (group U) with 9 patients and the BVR candidates (group B) with 8 patients. In group B, the median value of the Z-score of the minimum LVOT increased from -3.2 (range: − 4.1 ~ − 1.0) at pre-PAB to -2.8 (range: − 3.6 ~ − 0.3) at pre-arch repair with a significant difference (p = 0.012). In group U, the median value of the Z-score of the neo-AoV increased from 0.5 (range: − 1.0 ~ 1.7) at pre-bPAB to 1.2 (range: 0.2 ~ 1.9) at pre-arch repair with a significant difference (p < 0.01). The median value of the Z-score of the neo-AAo was also increased from 3.1 (range: 1.5 ~ 4.6) to 4.3 (range: 3.1 ~ 5.9) with a significant difference (p = 0.028). The growth of the LVOT for BVR candidates and SVOT for UVR candidates during the inter-stage between bPAB and arch repair was observed. These results suggest the potential advantage of bPAB in surgical strategies. Further research is needed to validate these findings and refine surgical approaches. [ABSTRACT FROM AUTHOR]

Background: Pulmonary hypertension (PH) is a life-threatening condition with high mortality, categorized into Group 1-5 by distinct etiologies. Fasudil, a potent vasodilator targeting RhoA/Rho kinase pathway, holds promise for diverse PH pathologies. However, a systematic evaluation of its clinical benefits remains elusive. Methods: We conducted a systematic search in several databases. Meta-analysis using odds ratio and mean difference was performed, with an assessment of studies' quality and pooled evidences. Results: Inclusion of 3269 Group-3 PH patients demonstrated that Fasudil increased effective events, forced expiratory volume in one second (FEV1), 6-minute walking distance (6MWD) and arterial partial pressure of oxygen (PaO2), and decreased mean pulmonary artery pressure (mPAP) and pulmonary artery systolic pressure (PASP); Inclusion of 197 Group-2 PH patients suggested that Fasudil increased 6MWD and PaO2, and decreased PASP. Subgroup analysis revealed no significant difference between 30 and 60 mg/day dosages of Fasudil, while administration durations and methods might affect its effectiveness in treating Group-3 PH patients. Conclusions: Our study favors the beneficial effects of Fasudil by enhancing FEV1, 6MWD and PaO2, and reducing mPAP and PASP on Group-3 PH patients, suggesting Fasudil as a viable treatment option and highlighting the need for further studies to inform healthcare policies. Protocol registration: identifier is CRD42022308947 [ABSTRACT FROM AUTHOR]

Bronchopulmonary dysplasia (BPD) is characterized by impaired lung alveolar and vascular growth. We investigated the hypothesis that neonatal exposure to hyperoxia leads to persistent BPD phenotype caused by decreased expression of liver kinase B1 (LKB1), a key regulator of mitochondrial function. We exposed mouse pups from Postnatal Day (P)1 through P10 to 21% or 75% oxygen. Half of the pups in each group received metformin or saline intraperitoneally from P1 to P10. Pups were killed at P4 or P10 or recovered in 21% O2 until euthanasia at P21. Lung histology and morphometry, immunofluorescence, and immunoblots were performed to detect changes in lung structure and expression of LKB1; downstream targets AMPK, PGC-1α, and electron transport chain (ETC) complexes; and Notch ligands Jagged 1 and delta-like 4. LKB1 signaling and in vitro angiogenesis were assessed in human pulmonary artery endothelial cells (exposed to 21% or 95% O2 for 36 hours. Levels of LKB1, phosphorylated AMPK, PGC-1α, and ETC complexes were decreased in lungs at P10 and P21 in hyperoxia. Metformin increased LKB1, phosphorylated AMPK, PGC-1α, and ETC complexes at P10 and P21 in pups exposed to hyperoxia. Radial alveolar count was decreased, and mean linear intercept increased in pups exposed to hyperoxia at P10 and P21; these were improved by metformin. Lung capillary density was decreased in hyperoxia at P10 and P21 and was increased by metformin. In vitro angiogenesis was decreased in human pulmonary artery endothelial cells by 95% O2 and was improved by metformin. Decreased LKB1 signaling may contribute to decreased alveolar and vascular growth in a mouse model of BPD. [ABSTRACT FROM AUTHOR]

Aims: A recent guideline presented by the ESC Congress in 2022 had indicated a novel therapy targeted at pulmonary artery hypertension, known as pulmonary artery denervation (PADN), which get inspired from a laboratorial trial that could lowering the pulmonary artery pressure through the intervention on the animals. Our aim is to conduct a network meta‐analysis to compare the efficacy and safety of PADN from six aspects with the current conventional therapies. Methods and results: According to the PRISMA guidance, databases including Ovid, ClinicalTrials.gov, Medline, Embase, and PubMed were searched from inception to 22 August 2023, along with a full assessment of the previous five meta‐analyses. Data were extracted and curated for Bayesian network meta‐analysis. The primary outcome was the change in the 6‐min walking distance (6MWD) from baseline with a secondary outcome called change in mean pulmonary artery pressure (mPAP) from baseline. The four safety outcomes included risk of clinical worsening, hospitalization, mortality and severe adverse events (SAEs). The comparison is structured on a contrast model based on 65 randomized controlled trials (RCTs) on PADN and the other conventional mainstream drugs. PADN had a better effect in improving 6MWD than Placebo (−77.76 m, 95% CI: −102.04 to −54.34 m), Macitentan (−65.32 m, 95% CI: −95.34 to −36.1 m), Bosentan (−64.5 m, 95% CI: −94.7 to −35.07 m), Iloprost (−62.66 m, 95% CI: −99.48 to −27.13 m), Oxygen (−62.42 m, 95% CI: −100.01 to −25.78 m), Treprostinil (−62.01 m, 95% CI: −89.04 to −35.61 m), Riociguat (−60.59 m, 95% CI: −86.11 to −35.98 m), Selexipag (−47.2 m, 95% CI: −85.61 to −10.19 m), Sildenafil (−44.92 m, 95% CI: −74.43 to −16.15 m), or Sitaxsentan (−39.53 m, 95% CI: −78.99 to −0.76 m). PADN had a better antihypertensive effect than placebo and showed statistical significant lower risks to induce clinical worsening and re‐hospitalization than treprostinil, riociguat, and placebo groups. No statistically significant difference in risk of mortality and severe adverse events was observed between PADN versus the other interventions. Conclusions: Compared with 16 types of conventional therapies and Placebo, PADN has advantage over nine single therapies and Placebo in improving 6MWD and appears to be better than two types of dual‐drug combined therapies while with no statistical significance. PADN shows a favourable antihypertensive effect on mPAP and has a lower risk to trigger clinical worsening or hospitalization, while its risk on mortality and severe adverse events is still inconclusive. [ABSTRACT FROM AUTHOR]

Aims: We report the results of a real‐world study based on heart failure (HF) patients' continuous remote monitoring strategy using the CardioMEMS system to assess the impact of this device on healthcare outcomes, costs, and patients' management and quality of life. Methods and results: We enrolled seven patients (69.00 ± 4.88 years; 71.43% men) with HF, implanted with CardioMEMS, and daily remote monitored to optimize both tailored adjustments of home therapy and/or hospital infusions of levosimendan. We recorded clinical, pharmacological, biochemical, and echocardiographic parameters and data on hospitalizations, emergency room access, visits, and costs. Following the implantation of CardioMEMS, we observed a 50% reduction in the total number of hospitalizations and a 68.7% reduction in the number of days in the hospital. Accordingly, improved patient quality of life was recorded with EQ‐5D (pre 58.57 ± 10.29 vs. 1 year post 84.29 ± 19.02, P = 0.008). Echocardiographic data show a statistically significant improvement in both systolic pulmonary artery pressure (47.86 ± 8.67 vs. 35.14 ± 9.34, P = 0.022) and E/e′ (19.33 ± 5.04 vs. 12.58 ± 3.53, P = 0.023). The Quantikine® HS High‐Sensitivity Kit determined elevated interleukin‐6 values at enrolment in all patients, with a statistically significant reduction after 6 months (P = 0.0211). From an economic point of view, the net savings, including the cost of CardioMEMS, were on average €1580 per patient during the entire period of observation, while the analysis performed 12 months after the implant vs. 12 months before showed a net saving of €860 per patient. The ad hoc analysis performed on the levosimendan infusions resulted in 315 days of hospital avoidance and a saving of €205 158 for the seven patients enrolled during the observation period. Conclusions: This innovative strategy prevents unplanned access to the hospital and contributes to the efficient use of healthcare facilities, human resources, and costs. [ABSTRACT FROM AUTHOR]

Aims: The initial bundle of cares strongly affects haemodynamics and outcomes in acute decompensated heart failure cardiogenic shock (ADHF‐CS). We sought to characterize whether 24 h haemodynamic profiling provides superior prognostic information as compared with admission assessment and which haemodynamic parameters best predict in‐hospital death. Methods and results: All patients with ADHF‐CS and with available admission and 24 h invasive haemodynamic assessment from two academic institutions were considered for this study. The primary endpoint was in‐hospital death. Regression analyses were run to identify relevant predictors of study outcome. We included 127 ADHF‐CS patients [65 (inter‐quartile range 52–72) years, 25.2% female]. Overall, in‐hospital mortality occurred in 26.8%. Non‐survivors were older, with greater CS severity. Among admission variables, age [odds ratio (OR) = 1.06; 95% confidence interval (CI): 1.02–1.11; Padj = 0.005] and CPIRAP (OR = 0.62 for 0.1 increment; 95% CI: 0.39–0.95; Padj = 0.034) were found significantly associated with in‐hospital death. Among 24 h haemodynamic univariate predictors of in‐hospital death, pulmonary elastance (PaE) was the strongest (area under the curve of 0.77; 95% CI: 0.68–0.86). PaE (OR = 5.98; 95% CI: 2.29–17.48; Padj < 0.001), pulmonary artery pulsatility index (PAPi, OR = 0.77; 95% CI: 0.62–0.92; Padj = 0.013) and age (OR = 1.06; 95% CI: 1.02–1.11; Padj = 0.010) were independently associated with in‐hospital death. Best cut‐off for PaE was 0.85 mmHg/mL and for PAPi was 2.95; cohort phenotyping based on these PaE and PAPi thresholds further increased in‐hospital death risk stratification; patients with 24 h high PaE and low PAPi exhibited the highest in‐hospital mortality (56.2%). Conclusions: Pulmonary artery elastance has been found to be the most powerful 24 h haemodynamic predictor of in‐hospital death in patients with ADHF‐CS. Age, 24 h PaE, and PAPi are independently associated with hospital mortality. PaE captures ventricular (RV) afterload mismatch and PAPi provides a metric of RV adaptation, thus their combination generates four distinct haemodynamic phenotypes, enhancing in‐hospital death risk stratification. [ABSTRACT FROM AUTHOR]

Aims: Novel echocardiographic parameters of right ventricular (RV) function, including speckle‐tracking‐derived, three‐dimensional, and RV–pulmonary artery coupling parameters, have emerged for the evaluation of pulmonary arterial hypertension (PAH). The relative role of these parameters in the risk stratification of PAH patients is unclear. We compared the performance of multiple RV parameters and sought to establish an optimal model for identifying the risk profile of patients with PAH. Methods and results: Comprehensive risk assessments were performed for 70 patients with PAH. The risk profile of every patient was determined based on the guideline recommendations. Conventional parameters, including fractional area change (FAC) and tricuspid annular plane systolic excursion (TAPSE), novel speckle‐tracking‐derived RV longitudinal strain (RVLS), and three‐dimensional RV ejection fraction (3D‐RVEF), were used to evaluate RV function. Pressure–strain loops were measured for the assessment of RV myocardial work, including RV global wasted work (RVGWW). RV–pulmonary artery coupling was assessed by indexing RV parameters to the estimated pulmonary artery systolic pressure (PASP). The median age was 34 (30–43) years, and 62 (88.6%) patients were female. Forty‐five patients were classified into the low‐risk group, while 25 patients were classified into the intermediate–high‐risk group. Most RV parameters could be used to determine the risk profile and exhibited significantly improved diagnostic performance after indexing to PASP (including FAC/PASP, TAPSE/PASP, and 3D‐RVEF/PASP). RVLS/PASP showed the best performance, with an area under the curve of 0.895. In multivariate analysis (Model 1), only RVGWW (>90.5 mmHg%), RVLS (> −16.7%), and TAPSE (<17.5 mm) remained significant (all P < 0.05). Model 1 outperformed every single RV parameter, with a significantly larger area under the curve (all P < 0.05). With PASP indexing in Model 2, RVLS/PASP > −0.275 [odds ratio (OR) 20.63, 95% confidence interval (CI) 4.62–92.11, P < 0.001] and RVGWW > 90.5 mmHg% (OR 6.17, 95% CI 1.37–27.76, P = 0.018) independently identified a higher risk profile. The addition of RVGWW to two models determined incremental value in identification (continuous net reclassification improvement 1.058, 95% CI 0.639–1.477, P < 0.001). Conclusions: The combination models for RV function outperformed any single parameter in identifying the risk profile of patients with PAH. Comprehensive assessment of RV–pulmonary artery coupling using multiparametric methods is clinically meaningful in patients with PAH. [ABSTRACT FROM AUTHOR]

Introduction: To date, there is no specific evidence or criteria for the selection of patients with PH and severe tricuspid insufficiency that can be initiated into correction of tricuspid valvulopathy. Tricuspid regurgitation is a risk marker independent of mortality in patients with pulmonary hypertension. The critical factor for the procedure’s success is to find the parameters to select patients so that they do not become just a futile act. Method: From the initial group of 271 patients, a final group of 123 patients were selected, all diagnosed with precapillary PH confirmed by catheterization and with tricuspid regurgitation by echocardiography. Patients were in groups 1 and 2 according to the 2022 Pulmonary Hypertension Guidelines. Patients with right to left shunt were not excluded. Results: In patients with severe precapillary PH, the sPAP/PAAT ratio was close to 1 (0.89 ± 0.43), while in patients with mild precapillary PH or in the postcapillary group, the sPAP/PAAT ratio was considerably lower (0.47 ± 0.20, p < 0.001). The average sPAP/PAAT of deceased patients was 0.76. Among the 68 deceased patients, 42 (61.70%) had severe tricuspid regurgitation. Conclusion: In our study, the average sPAP/PAAT ratio of the deceased patients with severe FTR was 0.76 mm Hg/ms; nevertheless, this knowledge could have a potential use but is not sufficient for full-informed qualification or disqualification for valve intervention, which requires specific TTVR-related data. [ABSTRACT FROM AUTHOR]

Background: Pulmonary artery hypertension (PAH) is a severe cardiovascular disease marked by a persistent increase in pulmonary artery resistance and pressure, leading to right ventricular strain, hypertrophy, and eventually right heart failure and death. Despite numerous available targeted therapies, the clinical needs for treating PAH remain unmet. Current treatments primarily aim to dilate pulmonary vessels rather than reverse pulmonary vascular remodeling, failing to offer a fundamental solution for PAH. Therefore, developing new therapies for this condition is urgently required. Summary: Recent research has highlighted the crucial role of non-coding RNAs (ncRNAs) in the occurrence and development of PAH. NcRNAs, such as long non-coding RNAs (lncRNAs), circular RNAs (circRNAs), microRNAs (miRNAs), and PIWI-interacting RNAs (piRNAs), are a class of transcripts that do not translate proteins but affect various diseases at different levels, including chromatin modification, transcription regulation, post-translational processes. Key Message: The current study delves into recent advancements in understanding how lncRNAs, circRNAs, miRNAs, and piRNAs contribute to the pathogenesis of PAH. This review addresses the existing research challenges and explores the potential of ncRNAs as both biomarkers and therapeutic targets, suggesting that ncRNAs may serve as valuable indicators and treatment options for the disease. [ABSTRACT FROM AUTHOR]

Mouse models are crucial for understanding pulmonary hypertension (PH) mechanisms and developing therapies, but existing mouse models under hypoxia only exhibit mild PH. To address this, we established a double‐hit model combining unilateral pneumonectomy (LPx) or left pulmonary artery ligation (LPAL) with hypoxia exposure in C57BL/6 mice. Our detailed haemodynamic and histological evaluations post‐surgery demonstrated pronounced elevations in right ventricular systolic pressure (RVSP) (LPAL: 41.1 ± 4.63 mmHg, P = 0.005; LPx: 38.4 ± 2.95 mmHg, P = 0.002; Sham: 32.1 ± 2.21 mmHg) and pulmonary vascular wall thickness (LPAL: 56.9 ± 3.34%, P = 0.02; LPx: 54.3 ± 4.65%, P = 0.04; Sham: 44.8 ± 3.76%) compared to hypoxia‐exposed sham‐operated controls, reflecting a more severe PH phenotype. These novel models, which exhibit haemodynamic alterations akin to the established hypoxia with SU5416‐induced PH model as per published data, could offer a substantial contribution to future PH research and therapeutic development. [ABSTRACT FROM AUTHOR]

Background: Measurement of tumor markers from peripheral venous blood is an emerging tool to assist in the early diagnosis of lung cancer. Samples from the pulmonary artery and pulmonary artery wedge position (trans-pulmonary samples) are accessible via right-heart catheterization and, by virtue of their proximity to lung tumors, may increase diagnostic yield. Case presentation: We report a case of a 64 year-old woman from whom trans-pulmonary samples were obtained and who was diagnosed 16 months later with recurrent metastatic small cell lung cancer. Carcinoembryonic antigen, cytokeratin fragment 21 − 1 (CYFRA), and human epididymis protein 4 (HE4) levels demonstrated increasing concentrations across the pulmonary circulation. These gradients exceeded the assays' coefficient of variation by several-fold. For CYFRA and HE4, pulmonary artery wedge concentrations exceeded peripheral venous levels by more than 10% and peripheral arterial levels were up to 8% higher than peripheral venous levels. Conclusions: Evaluating the feasibility and utility of trans-pulmonary tumor markers for lung cancer diagnosis in a larger cohort should be considered. The addition of a peripheral arterial sample to standard peripheral venous samples may be a more practical alternative. [ABSTRACT FROM AUTHOR]

Simple Summary: Contrast-enhanced computed tomography (CT) is the standard radiologic examination for evaluating mediastinal tumors. In equivocal cases, cine magnetic resonance imaging (cine-MRI) and magnetic resonance imaging (MRI)/T1-weighted spin echo sequences (T1TSE) may be additionally performed. We retrospectively analyzed patients undergoing surgical resection of mediastinal tumors (benign and malignant) with prior CT, cine-MRI, and T1TSE for (re-)evaluation and comparison to the intraoperative findings and postoperative histology reports, which were defined as a gold standard. Unclear CT cases were further investigated. A total of 47 patients were included in the study. Cine-MRI is of crucial benefit in unclear CT findings compared with T1TSE, especially when infiltration into the large central vessels and atria is suspected. Background/Objectives: Contrast-enhanced computed tomography (CT) is the standard radiologic examination for evaluating the extent of mediastinal tumors. If tumor infiltration into the large central thoracic vessels, the pericardium, or the myocardium is suspected, cine magnetic resonance imaging (cine-MRI) can provide additional valuable information. Methods: We conducted a retrospective study of patients with mediastinal tumors who were staged with CT, cine-MRI, and a T1-weighted turbo spin echo (T1TSE) prior to surgical resection. Imaging was re-evaluated regarding tumor infiltration into the pericardium, myocardium, superior vena cava, aorta, pulmonary arteries, and atria and compared with intraoperative findings and postoperative histopathological reports (gold standard). Unclear CT findings were further investigated. Results: Forty-seven patients (29 female and 18 male patients; median age: 58 years) met the inclusion criteria. Cine-MRI was able to predict infiltration of the aorta in 86%, pulmonary arteries in 85%, and atria in 80% of unclear CT cases. Aortic tumor infiltration in unclear CT cases was significantly more often correctly diagnosed with cine-MRI than with T1TSE sequence. Conclusions: Additional cine-MRI is of crucial benefit in unclear CT cases. We recommend performing cine-MRI if infiltration into the large central vessels and atria is suspected. T1TSE sequence is of very limited additional value. [ABSTRACT FROM AUTHOR]

BACKGROUND: Pulmonary hypertension (PH) is a major complication linked to adverse outcomes in heart failure with preserved ejection fraction (HFpEF), yet no specific therapies exist for PH associated with HFpEF (PH-HFpEF). We have recently reported on the role of skeletal muscle SIRT3 (sirtuin-3) in modulation of PH-HFpEF, suggesting a novel endocrine signaling pathway for skeletal muscle modulation of pulmonary vascular remodeling. METHODS: Using skeletal muscle-specific Sirt3 knockout mice (Sirt3skm-/-) and mass spectrometry-based comparative secretome analysis, we attempted to define the processes by which skeletal muscle SIRT3 defects affect pulmonary vascular health in PH-HFpEF. RESULTS: Sirt3skm-/- mice exhibited reduced pulmonary vascular density accompanied by pulmonary vascular proliferative remodeling and elevated pulmonary pressures. Comparative analysis of secretome by mass spectrometry revealed elevated secretion levels of LOXL2 (lysyl oxidase homolog 2) in SIRT3-deficient skeletal muscle cells. Elevated circulation and protein expression levels of LOXL2 were also observed in plasma and skeletal muscle of Sirt3skm-/- mice, a rat model of PH-HFpEF, and humans with PH-HFpEF. In addition, expression levels of CNPY2 (canopy fibroblast growth factor signaling regulator 2), a known proliferative and angiogenic factor, were increased in pulmonary artery endothelial cells and pulmonary artery smooth muscle cells of Sirt3skm-/- mice and animal models of PH-HFpEF. CNPY2 levels were also higher in pulmonary artery smooth muscle cells of subjects with obesity compared with nonobese subjects. Moreover, treatment with recombinant LOXL2 protein promoted pulmonary artery endothelial cell migration/proliferation and pulmonary artery smooth muscle cell proliferation through regulation of CNPY2-p53 signaling. Last, skeletal muscle--specific Loxl2 deletion decreased pulmonary artery endothelial cell and pulmonary artery smooth muscle cell expression of CNPY2 and improved pulmonary pressures in mice with high-fat diet--induced PH-HFpEF. CONCLUSIONS: This study demonstrates a systemic pathogenic impact of skeletal muscle SIRT3 deficiency in remote pulmonary vascular remodeling and PH-HFpEF. This study suggests a new endocrine signaling axis that links skeletal muscle health and SIRT3 deficiency to remote CNPY2 regulation in the pulmonary vasculature through myokine LOXL2. Our data also identify skeletal muscle SIRT3, myokine LOXL2, and CNPY2 as potential targets for the treatment of PH-HFpEF. [ABSTRACT FROM AUTHOR]

Background and Aims Based on retrospective studies, the 2022 European guidelines changed the definition of post-capillary pulmonary hypertension (pcPH) in heart failure (HF) by lowering the level of mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR). However, the impact of this definition and its prognostic value has never been evaluated prospectively. Methods Stable left HF patients with the need for right heart catheterization were enrolled from 2010 to 2018 and prospectively followed up in this multicentre study. The impact of the successive pcPH definitions on pcPH prevalence and subgroup [i.e. isolated (IpcPH) vs. combined pcPH (CpcPH)] was evaluated. Multivariable Cox regression analysis was used to assess the prognostic value of mPAP and PVR on all-cause death or hospitalization for HF (primary outcome). Results Included were 662 HF patients were (median age 63 years, 60% male). Lowering mPAP from 25 to 20 mmHg resulted in +10% increase in pcPH prevalence, whereas lowering PVR from 3 to 2 resulted in +60% increase in CpcPH prevalence (with significant net reclassification improvement for the primary outcome). In multivariable analysis, both mPAP and PVR remained associated with the primary outcome [hazard ratio (HR) 1.02, 95% confidence interval (CI) 1.00–1.03, P =.01; HR 1.07, 95% CI 1.00–1.14, P =.03]. The best PVR threshold associated with the primary outcome was around 2.2 WU. Using the 2022 definition, pcPH patients had worse survival compared with HF patients without pcPH (log-rank, P =.02) as well as CpcPH compared with IpcPH (log-rank, P =.003). Conclusions This study is the first emphasizing the impact of the new pcPH definition on CpcPH prevalence and validating the prognostic value of mPAP > 20 mmHg and PVR > 2 WU among HF patients. [ABSTRACT FROM AUTHOR]

Objective: Pulmonary artery hypertension (PAH) poses a significant challenge due to its limited therapeutic options and high mortality rates. The ACE2-Ang- (1-7)-Mas axis plays a pivotal role in regulating blood pressure and inhibiting myocardial remodeling. However, the precise mechanistic links between the ACE2-Ang-(1-7)-Mas axis and PAH remain poorly understood. This study aimed to elucidate the involvement of the ACE2-Ang-(1-7)-Mas axis in the development of PAH. Methods: PAH was induced in mice using Sugen5416/hypoxia, PAAT/PET ratio and PA were detected using cardiac ultrasound; inflammation related factors such as MCP-1, TNF, IL-10and IL-12p70 were detected in intestines using cytometric bead array (CBA) kits; histopathological and morphological changes in lung and intestinal tissues were assessed via HE staining and Masson staining to evaluate the progression of PAH. Immunohistochemistry and western blotting were employed to determine the expression levels of two tight junction proteins, occludin and ZO-1, in intestinal tissues. Additionally, 16SrRNA sequencing and non-targeted metabolomics by LC-MS/MS techniques were utilized to investigate the impact of the ACE2-Ang-(1-7)-Mas axis on microbial diversity and metabolomics of intestinal contents. Results: Activation of the ACE2-Ang-(1-7)-Mas axis improves heart function, reduces intestines inflammatory factors and ameliorates pathological and histological alterations in SuHx mice. This activation notably upregulated the expression of occludin and ZO-1 proteins in intestinal tissues and promoted the proliferation of SCFA-producing bacteria genera, such as g_Candidatus_ Saccharimonas. Furthermore, it enhanced the abundance of beneficial metabolites, including tryptophan and butyric acid. Conclusion: The findings suggest that modulation of the ACE2-Ang-(1-7)-Mas axis can alleviate PAH by regulating intestinal microbes and metabolites. These results highlight the potential of the ACE2-Ang-(1-7)-Mas axis as a promising therapeutic target for clinical management of PAH. [ABSTRACT FROM AUTHOR]

A fifteen-month-old Pembroke Welsh corgi with respiratory distress, exercise intolerance, and moderate regenerative anemia was referred to The Norwegian University of Life Sciences, Small Animal Hospital. Hematology revealed moderate regenerative anemia without evidence of hemolysis. Thoracic radiographs showed a generalized mixed interstitial to alveolar lung pattern and enlarged pulmonary arteries. Changes suggestive of moderate pulmonary hypertension were noted on echocardiography. Baermann fecal diagnostic flotation identified large numbers of Angiostrongylus vasorum larvae, and the AngioDetect serological antigen test was positive. The dog was treated with a two-week course with fenbendazole (51 mg/kg q24h po) and topical imidacloprid/moxidectin (250 mg/62.5 mg) and a one-week course with sildenafil (0.45 mg/kg q12h po). Complete clinical, clinicopathological and echocardiographic resolution was observed after only four weeks. Rapid improvement of echocardiographic abnormalities in cases with suspected pulmonary hypertension is not usually reported in cases with angiostrongylosis. Infection with A. vasorum should be considered in dogs with respiratory signs and bleeding tendencies, even in countries with no endemic history or reported cases. [ABSTRACT FROM AUTHOR]

Objective: This study aims to present the midterm outcomes of surgical correction of the anomalous left coronary artery from the pulmonary artery (ALCAPA). Methods: This is a retrospective study of patients undergoing anomalous origin of the LCA from the pulmonary artery repair between 2010 and 2019. Results: Forty-nine patients (20 boys and 29 girls) underwent ALCAPA repair. Patients were divided into two groups based on their age at ALCAPA repair: infant (< 1 year of age: n = 24) and non-infant (≧ 1 year of age: n = 25). Median age at time of repair was 23 months(7-60months). LCA reimplantation was performed in 47 patients, and Takeuchi repair was performed in 2 patients. Hospital mortality in the infant group was 8.2% (4 of 49). Infant group had significantly lower LVEF in pre-operation (p < 0.05), but there was not significantly different between the two groups about LVEF at discharge. The median follow-up duration was 43(18–85)months. The freedom from reoperation was not significantly different between two groups (infants vs. non-infants: 68.8% vs. 87.5% at 10 years; p = 0.096). Conclusions: Surgical treatment of ALCAPA had an excellent early and midterm outcomes. Left ventricular dysfunction in pre-operation was the main risk of mortality in-hospital. The freedom from reoperation did not differ significantly between infant group and non-infant group. [ABSTRACT FROM AUTHOR]