Risk Stratification and Prognosis of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Background:Current guidelines for managing pulmonary arterial hypertension(PAH)recommend a risk strati-fication approach.However,the applicability and accuracy of these strategies for PAH associated with congenital heart disease(PAH-CHD)require further validation.This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up.Additionally,new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients.Methods:This retrospective study included 126 PAH-CHD patients.Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s exact tests.Indepen-dent risk factors were identified using ordered logistic regression,while Kaplan-Meier and Cox proportional hazards regression analyses evaluated their impact on all-cause mortality.A new stratification model for the PAH-CHD population was constructed based on these analyses.Results:Significant survival differences across stratified risk groups were observed(p<0.001),validating the effectiveness of the simplified risk stratification method in PAH-CHD patients.Prothrombin activity was a strong independent predictor of adverse outcomes of PAH-CHD patients(Hazard ratio 0.95,p<0.001,C-index 0.70).A model combining N-terminal pro-brain natriuretic peptide,prothrombin activity,albumin,and right atrial area achieved an area under the curve of 0.89 and a C-index of 0.85.Conclusions:The simplified risk stratification method is applicable to PAH-CHD patients.Prothrombin activity is a strong independent predictor of adverse outcomes.A comprehensive risk stratification approach,incorporating both established and novel biomarkers,enhances accessibility and offers predictive efficacy during follow-up for PAH-CHD patients,comparable to established models.

Mufangji tang ameliorates pulmonary arterial hypertension through improving vascular remodeling,inhibiting inflammatory response and oxidative stress,and inducing apoptosis

Background:Mufangji tang(MFJT)is composed of Ramulus Cinnamomi,Radix Ginseng,Cocculus orbiculatus(Linn.)DC.,and Gypsum.In clinical settings,MFJT has been effectively employed in addressing a range of respiratory disorders,notably including pulmonary arterial hypertension(PAH).However,the mechanism of action of MFJT on PAH remains unknown.Methods:In this study,a monocrotaline-induced PAH rat model was established and treated with MFJT.The therapeutic effects of MFJT on PAH rat model were evaluated.Network pharmacology was conducted to screen the possible targets for MFJT on PAH,and the molecular docking between the main active components and the core targets was carried out.The key targets identified from network pharmacology were tested.Results:Results showed significant therapeutic effects of MFJT on PAH rat model.Analysis of network pharmacology revealed several potential targets related to apoptosis,inflammation,oxidative stress,and vascular remodeling.Molecular docking showed that the key components were well docked with the core targets.Further experimental validation results that MFJT treatment induced apoptosis(downregulated Bcl-2 levels and upregulated Bax levels in lung tissue),inhibited inflammatory response and oxdative stress(decreased the levels of IL-1β,TNF-α,inducible NOS,and malondialdehyde,and increased the levels of endothelial nitric oxide synthase,nitric oxide,glutathione and superoxide dismutase),reduced the proliferation of pulmonary arterial smooth muscle cells(downregulated ET-1 andβ-catenin levels and ERK1/2 phosphorylation,increased GSK3βlevels).Conclusion:Our study revealed MFJT treatment could alleviate PAH in rats via induction of apoptosis,inhibition of inflammation and oxidative stress,and the prevention of vascular remodeling.

Arterial Duct Stenting Versus Modified Blalock-Taussig Shunt in Patient with Ductal-Dependent Pulmonary Circulation: Systematic Review & Meta-Analysis

Objective:Patients with ductal-dependent pulmonary circulation require alternative bloodflow to provide and maintain adequate oxygenation.Modified Blalock-Taussig Shunt(MBTS)has been the standard for providing such a result.Currently,less invasive methods such as Arterial Duct(AD)stenting have been performed as alter-natives.This study aims to compare the outcome of AD stenting and MBTS.Method:Systematic research was performed in online databases using the PRISMA protocol.The outcomes measured were 30-day mortality,com-plication,unplanned intervention,oxygen saturation,duration of hospital,and ICU length of stay.Any compara-tive study provided with full text is included.The outcome of each study was analyzed using a trandom effects model with relative risk and mean difference as the effect size.Bias risk assessment was conducted using the New-castle-Ottawa Scale.All analyses were performed using Review Manager 5.4.1.Result:A total of 11 studies with 3154 samples included in this study.There is no significant difference in 30-day mortality between the two groups(p-value=0.10).However,there is significantly less complication(RR 0.53[0.35,0.82];p-value=0.004)and unplanned intervention(RR 0.59[0.38,0.92];p-value=0.02)in the AD stent group.Comparison of the Nakata index showed no significant difference(p-value=0.88).Post-operative oxygen saturation was measured signifi-cantly higher in the AD stenting(MD 1.80[0.85,2.74];p-value=0.0002).However,AD stent group shows sig-nificantly lower long-term oxygen saturation(MD-8.43[-14.38,-2.48];p-value=0.005).Both hospital and ICU length of stay was significantly shorter in the AD stent group(MD-8.30[-11.13,-5.48];p-value<0.00001;MD-5.09[-7.79,-2.38];p-value=0.0002).Conclusion:AD stenting provides comparable outcomes relative to MBTS as it provides less complication and unplanned intervention and higher post-procedural O2 saturation.However,MBTS proved its superiority in maintaining higher long-term oxygen saturation and still became the preferred option to manage complex cases where stenting is either challenging or unsuccessful.

Migration of a Progestin Implant into a Pulmonary Artery: Observation of a Case at the Reference Health Center of Commune III of the District of Bamako in Mali

It was a 36-year-old patient, 5th Pregnancy, 5th Delivery, 4 live children, and I deceased, had a consultation in the department for the removal of implants. Questioning revealed that she had had the implant three years ago without medical follow-up. It was in view of the difficulties in extracting the capsule that the chest X-ray and CT scan carried out concluded that the implant had migrated into a branch of the left pulmonary artery. Therapeutic abstention has been the attitude of cardiovascular surgeons.

Removal of a pulmonary artery foreign body during pulse ablation in a patient with atrial fibrillation:A case report

BACKGROUND Foreign bodies in the pulmonary circulation have been documented in the literature and are typically caused by interventional procedures.However,reports of pulmonary artery foreign bodies during femoral vein puncture are rare,and there is no description of this complication from the guidewire surface flows into the pulmonary artery during a pulse ablation in a patient with atrial fibrillation.CASE SUMMARY We described a case in which a linear foreign body suddenly appeared on fluoroscopy image during pulsed ablation of atrial fibrillation.Multiposition angiography showed that the foreign body was currently lodged in the pulmonary artery but was hemodynamically stable.We then chose to use an interventional approach to remove the foreign body from the pulmonary artery.This foreign body was subsequently confirmed to be from the hydrophilic coating of the guidewire surface.This may be related to the difficulties encountered during the puncture of the femoral vein.This is a rare and serious complication of femoral vein puncture.Therefore,we reported this case in order to avoid a similar situation.CONCLUSION Mismatches between interventional devices from different manufacturers used for femoral venipuncture may result in pulmonary artery foreign bodies.

Inhibitory Effect of PPARδAgonist GW501516 on Proliferation of Hypoxia-induced Pulmonary Arterial Smooth Muscle Cells by Regulating the mTOR Pathway

Objective This study aimed to investigate the effects of the peroxisome proliferator-activated receptorδ(PPARδ)agonist GW501516 on the proliferation of pulmonary artery smooth muscle cells(PASMCs)induced by hypoxia,in order to search for new drugs for the treatment and prevention of pulmonary vascular remodeling.Methods PASMCs were incubated with different concentrations of GW501516(10,30,100 nmol/L)under the hypoxic condition.The proliferation was determined by a CCK-8 assay.The cell cycle progression was analyzed by flow cytometry.The expression of PPARδ,S phase kinase-associated protein 2(Skp2),and cell cycle-dependent kinase inhibitor p27 was detected by Western blotting.Then PASMCs were treated with 100 nmol/L GW501516,100 nmol/L mammalian target of rapamycin(mTOR)inhibitor rapamycin and/or 2µmol/L mTOR activator MHY1485 to explore the molecular mechanisms by which GW501516 reduces the proliferation of PASMCs.Results The presented data demonstrated that hypoxia reduced the expression of PPARδin an oxygen concentration-and time-dependent manner,and GW501516 decreased the proliferation of PASMCs induced by hypoxia by blocking the progression through the G0/G1 to S phase of the cell cycle.In accordance with these findings,GW501516 downregulated Skp2 and upregulated p27 in hypoxia-exposed PASMCs.Further experiments showed that rapamycin had similar effects as GW501516 in inhibiting cell proliferation,arresting the cell cycle,regulating the expression of Skp2 and p27,and inactivating mTOR in hypoxia-exposed PASMCs.Moreover,MHY1485 reversed all the beneficial effects of GW501516 on hypoxia-stimulated PASMCs.Conclusion GW501516 inhibited the proliferation of PASMCs induced by hypoxia through blocking the mTOR/Skp2/p27 signaling pathway.

NR4A1 enhances glycolysis in hypoxia-exposed pulmonary artery smooth muscle cells by upregulating HIF-1αexpression

Background:Pulmonary arterial hypertension(PAH)is a chronic and progressive disease that is strongly associated with dysregulation of glucose metabolism.Alterations in nuclear receptor subfamily 4 group A member 1(NR4A1)activity alter the outcome of PAH.This study aimed to investigate the effects of NR4A1 on glycolysis in PAH and its underlying mechanisms.Methods:This study included twenty healthy volunteers and twenty-three PAH patients,and plasma samples were collected from the participants.To mimic the conditions of PAH in vitro,a hypoxia-induced model of pulmonary artery smooth muscle cell(PASMC)model was established.The proliferation of PASMCs was assessed using CCK8 assays.Results:Levels of NR4A1,hypoxia-inducible factor-1α(HIF-1α),and various glycolysis-related enzymes were measured.In addition,extracellular glucose and lactate production were assessed.The interaction between NR4A1 and HIF-1αwas evaluated by co-immunoprecipitation assays.Levels of NR4A1 and HIF-1αwas increased in PAH patients,and exposure to hypoxia resulted in increased levels of NR4A1 and HIF-1αin PASMCs.NR4A1 interacted with HIF-1α.NR4A1 overexpression enhanced hypoxia-induced expression of HIF-1α,GLUT1,PKM2,HK2,and CD36,decreased glucose levels,increased lactate levels and promoted hypoxic PASMC viability.Conversely,silencing NR4A1 decreased hypoxia-induced expression of HIF-1α,GLUT1,PKM2,HK2,and CD36,promoted glucose production,reduced lactate levels and inhibited hypoxic PASMC viability.Furthermore,overexpression of HIF-1αreversed the regulation of glycolysis caused by NR4A1 knockdown.Conclusion:NR4A1 enhances glycolysis in hypoxia-induced PASMCs by upregulating HIF-1α.Our findings indicate that the management of NR4A1 activity may be a promising strategy for PAH therapy.

“Treat-Repair-Treat”:Management of Left Main Coronary Compression by a Pulmonary Artery Aneurysm in a Patient with Atrial Septal Defect and Significant Pulmonary Hypertension

Left main coronary compression syndrome(LMCS)may complicate pulmonary artery aneurysms(PAA),usually developed in the context of pulmonary arterial hypertension(PAH).We report the case of a 51-year-old female patient with an atrial septal defect(unsuitable for device closure)complicated by a PAA generating a 90%left main stenosis.The significant PAH held us back from immediate surgery.After specific dual PAH-targeted therapy(sildenafil and bosentan),the atrial septal defect could be closed with a unidirectional valved patch;the PAAinduced LMCS was treated by reductive arterioplasty.The postoperative course was uneventful.Follow-up showed clinical improvement,but PAH treatment was still needed.After three months,coronary angiography showed only an insignificant residual left main stenosis,proving that reductive pulmonary arterioplasty was effective in treating LMCS.Any PAA requires further evaluation for LMCS,a dangerous but treatable complication.The“treat-repair-treat”strategy and shunt-closure with a unidirectional valved patch can both improve surgical prospects of LMCS with shunt-related PAH.

Long-Term Outcomes of Systemic-to-Pulmonary Artery Shunt in Patients with Functional Single Ventricle and Heterotaxy Syndrome

Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpatients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiringearly modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonaryblood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank:p = 0.04), but the statistical significance disappeared at 20 years (log rank: p = 0.18). Among 31 patients whounderwent SPS, 11 (35%) had extracardiac total anomalous pulmonary venous connection (TAPVC). The survivalrate after SPS was 80% at 10 years. Cox regression analysis showed that extracardiac TAPVC (hazard ratio6.44, 95% confidence interval 1.23–33.7, p = 0.03) and pulmonary venous obstruction (PVO) at TAPVC repair(hazard ratio 11.2, 95% confidence interval 2.13–58.5, p = 0.004) were significantly associated with death. In25 patients who underwent bidirectional cavopulmonary shunt (BCPS), surgical interventions on the pulmonaryartery (PA) were performed after SPS in 7 of 9 patients with PA coarctation, 3 of 4 with non-confluent PAs, and4 of 12 with normal PAs. At SPS, primary central PA plasty was performed in three patients with PA coarctationand 2 with non-confluent PAs. There was no significant difference in the PA index before BCPS between patientswith and without primary central PA plasty (p = 0.49). Among 20 patients who underwent total cavopulmonaryconnection (TCPC), adverse events occurred in 7 (35%) patients, including death in 1 (5%), intervention for pulmonaryarteriovenous malformation (PAVM) in 3 (15%), and surgical intervention for PVO in 3 (15%). TheB-type natriuretic peptide concentration was significantly higher in patients with than without adverse events(p = 0.02). The adverse event-free survival rate after TCPC was 69% at 10 years. Conclusion: ExtracardiacTAPVC and PVO at TAPVC repair were significantly associated with death after SPS in patients who had heterotaxysyndrome with a single ventricle. Surgical interventions on the PA were frequently required after SPS inpatients with PA coarctation or non-confluent PAs. Although satisfactory survival was achievable after TCPC,late-onset PAVM and PVO remain concerns.

Rutaecarpine attenuates monocrotaline-induced pulmonary arterial hypertension in a Sprague-Dawley rat model

Background:Pulmonary arterial hypertension presents with obliterative remodeling of the pulmonary arteries and progressive elevation of pulmonary vascular resistance,which increase the risk of right ventricular failure and death.It has been reported in previous studies that rutaecarpine plays a crucial role in anti-inflammatory and antioxidant activities,which may help regulate cell apoptosis and cell proliferation.The purpose of this study was to determine the effects of rutaecarpine in the rat model of monocrotaline-induced pulmonary hypertension.Methods:We induced pulmonary arterial hypertension in adult Sprague-Dawley rats by injecting monocrotaline(60 mg/kg)and then treated with rutaecarpine(40 mg/kg·d)or sildenafil(30 mg/kg·d)(positive control).Subsequently,pulmonary function,inflammation,cytokines and pulmonary vascular remodeling or proliferation were assessed.Results:Rutaecarpine was found to improve monocrotaline-induced mean pulmonary artery pressure,cardiac index,right heart index,right ventricular hypertrophy index,pulmonary artery remodeling and pulmonary function.reverse transcription-quantitative polymerase chain reaction demonstrated a decrease in tumor necrosis factor-α,interleukin-6 and interleukin-1β,whereas western blots a significantly decrease in the expression of nuclear factor kappa-B,endothelin-1,extracellular signal-regulated kinases 1/2,B cell lymphoma-2,Beclin1 and microtubule-associated protein1 light chain 3-II protein,and increase in the expression of Bax,caspase-3 and p62 protein.Conclusion:Rutaecarpine attenuated pulmonary arterial hypertension by inhibiting inflammation,oxidative stress,cell proliferation and autophagy,while promoting apoptosis.

Giant-Size Main Pulmonary Artery Aneurysm in an Adult Patient with Ebstein Anomaly and Dextrocardia

Main pulmonary artery aneurysms are rare,mostly asymptomatic and discovered accidentally.The main pulmonary artery aneurysms may be idiopathic or secondary to underlying diseases such as pulmonary hypertension,Behcet’s disease,connective tissue disorders,congenital heart disease,vasculitis,syphilis,tuberculosis and endocarditis.There are some indices that dextrocardia is associated with anomalies of the pulmonary arteries and pulmonary valve.A rare occurrence of main pulmonary artery aneurysms results in a lack of recommendations,so the remaining challenges are whether main pulmonary artery aneurysms should be treated,how,and when.The decision on surgical intervention or conservative treatment of the main pulmonary artery aneurysms depends on its size,etiology and accompanying diseases and includes a multidisciplinary heart team.Our case of the main pulmonary artery aneurysm and pulmonary valve abnormality associated with Ebstein anomaly and dextrocardia in a 67-year-old male patient brings causality considerations and treatment options in such a unique condition.

Pulmonary artery aneurysm protruding into the bronchus as an endobronchial mass:A case report

BACKGROUND Pulmonary artery(PA)aneurysms are usually diagnosed radiographically and present as small or large lesions resembling inflammation or a neoplasm on chest radiography.It has rarely been reported as an endobronchial mass.CASE SUMMARY We report the case of a 64-year-old man who presented with recurrent hemoptysis.Bronchoscopy revealed a tumorous protrusion blocking the right middle lobe bronchus,which was confirmed to be a PA aneurysm using endobronchial ultrasound bronchoscopy and computed tomography angiography.CONCLUSION Although endobronchial PA aneurysms are rare,bronchoscopists need to add this lesion to the list of endobronchial masses for which a biopsy is to be assiduously avoided.

Pulmonary arterial hypertension confirmed by right heart catheterization following COVID-19 pneumonia: A case report and review of literature

BACKGROUND Pulmonary arterial hypertension(PAH)is a disease of the arterioles resulting in an increased resistance in pulmonary circulation with associated high pressures in the pulmonary arteries,causing irreversible remodeling of the pulmonary arterial walls.Coronavirus disease 2019(COVID-19)has been associated with development of new onset PAH in the literature leading to symptoms of dyspnea,cough and fatigue that persist in spite of resolution of acute COVID-19 infection.However,the majority of these cases of COVID related PAH were diagnosed using echocardiographic data or via right heart catheterization in mechanically ventilated patients.CASE SUMMARY Our case is the first reported case of COVID related PAH diagnosed by right heart catheterization in a non-mechanically ventilated patient.Right heart catheterization has been the gold standard for diagnosis of pulmonary hypertension.Our patient had right heart catheterization four months after her initial COVID-19 infection due to persistent dyspnea.CONCLUSION This revealed new onset PAH that developed following her infection with COVID-19,an emerging sequela of the infection.

Establishment of adult right ventricle failure in ovine using a graded, animal-specific pulmonary artery constriction model

Background: Right ventricle failure(RVF) is associated with serious cardiac and pulmonary diseases that contribute significantly to the morbidity and mortality of patients. Currently, the mechanisms of RVF are not fully understood and it is partly due to the lack of large animal models in adult RVF. In this study, we aim to establish a model of RVF in adult ovine and examine the structure and function relations in the RV.Methods: RV pressure overload was induced in adult male sheep by revised pulmonary artery constriction(PAC). Briefly, an adjustable hydraulic occluder was placed around the main pulmonary artery trunk. Then, repeated saline injection was performed at weeks 0, 1, and 4, where the amount of saline was determined in an animal-specific manner. Healthy, age-matched male sheep were used as additional controls. Echocardiography was performed bi-weekly and on week 11 post-PAC, hemodynamic and biological measurements were obtained.Results: This PAC methodology resulted in a marked increase in RV systolic pressure and decreases in stroke volume and tricuspid annular plane systolic excursion, indicating signs of RVF. Significant increases in RV chamber size, wall thickness, and Fulton's index were observed. Cardiomyocyte hypertrophy and collagen accumulation(particularly type III collagen) were evident, and these structural changes were correlated with RV dysfunction.Conclusion: In summary, the animal-specific, repeated PAC provided a robust approach to induce adult RVF, and this ovine model will offer a useful tool to study the progression and treatment of adult RVF that is translatable to human diseases.

Debulking treatment with CT-guided percutaneous radiofrequency ablation and hepatic artery infusion of floxuridine improves survival of patients with unresectable pulmonary and hepatic metastases of colorectal cancer

The survival of most patients with both unresectable hepatic and pulmonary metastases of colorectal cancer is poor. In this retrospective study, we investigated the efficacy of computed tomography(CT)-guided radiofrequency ablation(RFA) and systemic chemotherapy plus hepatic artery infusion of floxuridine(HAIFUDR). Sixty-one patients were selected from 1,136 patients with pulmonary and hepatic metastases from colorectal cancer. Patients were treated with RFA and systemic chemotherapy plus HAI-FUDR(ablation group, n = 39) or systemic chemotherapy plus HAI-FUDR(FUDR group, n = 22). Patients in the two groups were matched by sex, age, number of metastases, and calendar year of RFA or FUDR. Survival data were evaluated by using univariate and multivariate analyses. Clinical characteristics were comparable between the two groups. All patients in the ablation group underwent RFA and chemotherapy. Median follow-up was 56.8 months. The 1-, 3-, and 5-year overall survival(OS) rates were 97%, 64%, and 37%, respectively, for the ablation group, and 82%, 32%, and 19%, respectively, for the FUDR group. The 1-, 3-, and 5-year survival rates after metastasis were 97%, 49%, and 26% for the ablation group, and 72%, 24%, and 24% for the FUDR group, respectively. The median OS times were 45 and 25 months for the ablation and FUDR groups, respectively. In the multivariate analysis, treatment allocation was a favorable independent prognostic factor for OS(P = 0.001) and survival after metastasis(P = 0.009). These data suggest that the addition of RFA to systemic chemotherapy plus HAI-FUDR improves the survival of patients with both unresectable hepatic and pulmonary metastases from colorectal cancer.

Effects of Combined Inhalation of Budesonide,Formoterol,and Tiotropium Bromide on Arterial Blood Gas and Pulmonary Function Indexes in Patients with Chronic Obstructive Pulmonary Disease

Objective:To analyze the effect of combined inhalation of budesonide formoterol and tiotropium bromide on arterial blood gas and pulmonary function indexes in patients with chronic obstructive pulmonary disease(COPD).Methods:100 patients with COPD treated from January to December 2022 were selected as observation objects,and were divided into a control group(n=50,in which budesonide and formoterol were administered)and an experimental group(n=50,the treatment drug was budesonide formoterol combined with tiotropium bromide)according to the computer grouping method,and compared the treatment results.Results:(i)Before treatment,there was no difference in the partial pressure of carbon dioxide and partial pressure of oxygen between the control group and the experimental group(P>0.05);after treatment,the partial pressure of carbon dioxide and partial pressure of oxygen in the experimental group were higher than those in the control group,with significant differences(P<0.05).(ii)Before treatment,there was no difference in forced vital capacity(FVC),forced expiratory volume in one second(FEV1),FEV1/FVC between the control group and the experimental group(P>0.05);after treatment,the FVC,FEV1,and FEV1/FVC in the experimental group were significantly higher than those in the control group(P<0.05).(iii)There was no difference in the levels of CRP,IL-6,and TNF-αbetween the control group and the experimental group(P>0.05);after treatment,the levels of CRP,IL-6,and TNF-αin the experimental group were lower than those in the control group,with significant differences(P<0.05).(iv)Compared to the total incidence of adverse reactions in the control group(28.00%),the incidence of total adverse reactions in the experimental group was lower at 10.00%,and the difference was significant(P<0.05).Conclusion:The combined inhalation of budesonide and formoterol with tiotropium bromide has demonstrated a clear therapeutic efficacy and safety in treating chronic obstructive pulmonary disease.This treatment approach effectively enhances arterial blood gas levels and lung function,showing promising potential for widespread application.

Intervention control of aerobic exercise in maintaining quality of life and pulmonary hypertension in hemodialysis patients

BACKGROUND Pulmonary hypertension is a serious complication in the treatment of maintenance hemodialysis patients,which seriously affects the quality of life of patients and threatens their life safety.Prevention,treatment and improvement of pulmonary hypertension are of great significance to improve the quality of life of patients.AIM To investigate the intervention and control of pedal-powered bicycle in maintaining quality of life and pulmonary hypertension in hemodialysis patients.METHODS 73 patients with maintenance hemadialysis combined with pulmonary arterial hypertension at a hemodialysis center in a certain hospital from May 2021 to May 2022 are selected.Patients are divided into two groups,37 cases in the control group(group C)and 36 cases in the intervention group(group I).Patients are divided into two groups,group C is treated with oral administration of betaglandin sodium combined with routine nursing care.Based on group C,group I conducts power cycling exercises.RESULTS After treatment,group I patients had higher muscle strength,36-Item Short Form Health Survey scores,and Kidney Disease Targets Areas scores;The 6-minute walk distance test index level was higher and the Borg score was lower;The group I had lower systolic blood pressure,greater vital capacity,higher positive emotion,lower systolic pulmonary artery pressure index level,higher arterial partial oxygen pressure level,lower pulmonary vascular resistance index level,and higher blood oxygen saturation level[158.91±11.89 vs 152.56±12.81,1795.01±603.18 vs 1907.20±574.15,24.00(22.00,29.00)vs 24.00(22.00,28.00),P<0.001].CONCLUSION Aerobic exercise combined with Western medicine treatment can effectively improve patients'pulmonary hypertension,alleviate their negative emotions,and enable them to achieve a higher level of quality of life.

Osthole attenuates pulmonary arterial hypertension by modulation of phospholipid metabolism

OBJECTIVE Pulmonary arterial hypertension(PAH)is a malignant pulmonary vascular disease lacking efficacy therapeutics.Therefore,it urgently needs to develop safe and effective drugs for PAH treatment.Osthole derived from Cnidium monnieri(L.)Cusson(Shechuangzi)or Angelica pubescens Maxim(Duhuo)has the capacity to alleviate PAH by decreasing pulmonary arterial pressure and alleviating pulmonary vascular remodeling in rats,which is a candidate drug for the prevention of PAH,but the underlying modulatory mechanism is still unclear.Our study aims at investigating the metabolic modulatory mechanism of osthole against PAH employing functional metabolomics strategy.METHODS PAH model rats were successfully established with MCT,following osthole administration,then functional metabolomics based on untargeted metabolomics assay,targeted lipidomics analysis,qRT-PCR,Western blotting and ELISA were performed to investigate the modulatory mechanism of osthole against pulmonary arterial pressure and pulmonary vascular remodeling in PAH.RESULTS Untargeted metabolomics results found that sphingosine 1-phosphate(S1P)was the differential metabolites characterized PAH and reversed by osthole treatment.S1P is a crucial sphingolipid metabolite catalyzed by sphingosine kinases1(Sphk1)and functions as promoting PASMCs proliferation contributing to pulmonary vascular remodeling and pulmonary arterial pressure increase.We revealed that osthole reversed high level of S1P by modulating metabolic enzyme Sphk1 via inactivating microRNA-21-PI3K/Akt/mTOR signal pathway to decrease pulmonary arterial pressure in rats with PAH.Then,targeted phospholipid metabolomics results uncovered that decadienyl-L-carnitine(C10:2)was the differential metabolite characterized PAH and corrected by osthole treatment in rat with PAH.C10:2 is the intermediate metabolite of fatty acid oxidation(FAO),and C10:2 accumulation indicated mitochondrial dysfunction and FAO increase.CONCLUSION Osthole could block lipid metabolic reprogramming through functional modulating the expression of fatty acid translocase,fatty acid synthase,phospholipase A2,carnitine palmitoyltransferase 1A to inhibit C10:2,thus to improve mitochondrial dysfunction and inhibit utilizing lipid to biosynthesize necessary essence for pulmonary artery smooth muscle cells(PASMCs)proliferation.Moreover,we delineated that C10:2 and metabolic reprogramming enzymes were modulated by miRNA-22-3p which was involved in PASMCs proliferation and pulmonary vascular remodeling.Therefore,osthole inhibited miRNA-22-3p mediated lipid metabolic reprogramming to ameliorate pulmonary vascular remodeling.

Intralobar Pulmonary Sequestration with Rete Mirabile as Part of Its Systemic Artery

Intralobar sequestration (ILS) is a complex lung lesion where part of a lower lobe lacks communication with the tracheobronchial tree and receives an aberrant arterial blood supply from the systemic circulation. That systemic artery is usually large, originating from the thoracic aorta or the abdominal aorta and its upper branches. We describe a case of ILS where a large systemic artery coming up from below the diaphragm is formed by the convergence of many very small serpiginous arteries coming up from the area of the celiac trunk and common hepatic artery: a kind of “rete mirabile”. This peculiar morphology was not described before.

Left Pulmonary Artery Sling Associated with Patent Ductus Arteriosus and Atrial Septal Defect: Evaluation with Multidetector CT

We report a case of left pulmonary artery sling associated with patent ductus arteriosus and atrial septal defect in a 21-month-old child. 256-slice MDCT provides valuable information, such as abnormal origin of the left pulmonary artery, the relationship between pulmonary artery and airway, the diameter of the patent ductus artery and atrial septal defect. The information is helpful in diagnosis, pre-operative evaluation and post-operative follow-up of LPS.