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 w...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.展开更多
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 allpat...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.展开更多
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 vei...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.展开更多
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 endobr...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.展开更多
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...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.展开更多
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(NR4...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.展开更多
Takayasu arteritis(TA)is a rare systemic vasculitis of the aorta and its primary branches,which usually occurs in young women.Due to its insidious onset and lack of specific symptoms,this disease can be easily misdiag...Takayasu arteritis(TA)is a rare systemic vasculitis of the aorta and its primary branches,which usually occurs in young women.Due to its insidious onset and lack of specific symptoms,this disease can be easily misdiagnosed or missed.Approximately 50%of the patients having TA with pulmonary artery involvement develop pulmonary hypertension(PH).The 3-year survival rate among patients with TA-related PH is lower than that among patients with TA alone.Early balloon pulmonary angioplasty(BPA)can improve the clinical symptoms and survival of patients with stable TA.To the best of our knowledge,this is the first case reported in the English literature in which a“Guidezilla”catheter was used during BPA to treat stenosis and occlusion of the pulmonary artery caused by Takayasu arteritis(TA).展开更多
BACKGROUND Uterine intravenous leiomyomatosis is defined as leiomyoma tissue invading the vein outside the leiomyoma.Reports of extension to the right pulmonary artery are relatively rare.CASE SUMMARY We describe a 31...BACKGROUND Uterine intravenous leiomyomatosis is defined as leiomyoma tissue invading the vein outside the leiomyoma.Reports of extension to the right pulmonary artery are relatively rare.CASE SUMMARY We describe a 31-year-old woman with a benign leiomyoma that extended into the right ventricular lumen,causing mechanical obstruction and corresponding clinical symptoms.Tumors located in the pulmonary artery can cause pulmonary artery obstruction.After diagnosis,surgical treatment should be performed as soon as possible.CONCLUSION In this case,the uterine leiomyoma extended to the right pulmonary system,which is clinically rare.展开更多
BACKGROUND Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery,which has a low incidence rate and is easily misdiagnosed as pulmonary embolism.There is no st...BACKGROUND Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery,which has a low incidence rate and is easily misdiagnosed as pulmonary embolism.There is no standard protocol for the treatment of primary intimal sarcoma of the pulmonary artery.CASE SUMMARY This study reports a patient with primary intimal sarcoma of the pulmonary artery who was admitted to our hospital in 2017.The clinical characteristics,diagnosis,treatment and outcome of the patient were retrospectively analyzed.The patient was a Chinese Han male aged 44 years.He had three consecutive episodes of syncope,and was thus admitted to a local hospital.Computed tomography pulmonary angiography showed multiple lesions with abnormal densities in the pulmonary trunk,left pulmonary artery,mediastinum and pericardium,which were consistent with recurrence after tumor resection.He underwent surgery,and was pathologically diagnosed with intimal sarcoma of the pulmonary artery.He relapsed 3 mo after surgery,and apatinib was administered.His condition was stable after 4 mo,with tolerable and controllable adverse reactions.He subsequently died 19 mo after surgery.CONCLUSION Primary intimal sarcoma of the pulmonary artery has no specific clinical or imaging manifestations.The diagnosis of this disease depends on histopathology and immunohistochemistry,and has a poor clinical prognosis.Surgical treatment is currently a favorable option for primary intimal sarcoma of the pulmonary artery,and targeted therapy may provide new insights for the development of effective treatment methods.展开更多
BACKGROUND Since 1923,only a few hundred cases of pulmonary arterial sarcoma(PAS)have been reported.It is easy for PAS to be misdiagnosed as pulmonary thromboembolism,which makes treatment difficult.The median surviva...BACKGROUND Since 1923,only a few hundred cases of pulmonary arterial sarcoma(PAS)have been reported.It is easy for PAS to be misdiagnosed as pulmonary thromboembolism,which makes treatment difficult.The median survival time without surgical treatment for PAS is only 1.5-3 mo.Echocardiography is widely used in screening for pulmonary artery space-occupying lesions in patients with chest pain,dyspnea,and cough;furthermore,it is typically considered the first imaging examination for patients with PAS.CASE SUMMARY In May 2017,a 39-year-old male patient experienced chest pain with no particular obvious cause.At that time,the cause was thought to be pulmonary embolism.In July 2017,positron emission tomography–computed tomography revealed spaceoccupying lesions in the right lung and multiple metastases in both lungs.The lesions of the right lung were biopsied,and pathology revealed undifferentiated sarcoma.Chemotherapy had been performed since July 2017 in another hospital.In December 2019,the patient was admitted to our hospital for the sake of CyberKnife treatment.Echocardiography suggested:(1)A right ventricular outflow tract(RVOT)solid mass of the main pulmonary artery;and(2)mild pulmonary valve regurgitation.Ultrasonography showed the absence of a thrombus in the deep veins of either lower limb.CONCLUSION PAS is a single,central space-occupying lesion involving the RVOT and pulmonary valve.Echocardiography of PAS has its own characteristics.展开更多
Cigarette smoking contributes to the development of pulmonary artery hypertension(PAH).As the basic pathological change of PAH,pulmonary vascular remodeling is considered to be related to the abnormal proliferation of...Cigarette smoking contributes to the development of pulmonary artery hypertension(PAH).As the basic pathological change of PAH,pulmonary vascular remodeling is considered to be related to the abnormal proliferation of pulmonary artery smooth muscle cells(PASMCs).However,the molecular mechanism underlying this process remains not exactly clear.The aim of this research was to study the molecular mechanism of PASMCs proliferation induced by smoking.Human PASMCs(HPASMCs)were divided into 6 groups:0%(control group),cigarette smoking extract(CSE)-treated groups at concentrations of 0.5%,1%,2%,5%,10%CSE respectively.HPASMCs proliferation was observed after 24 h.HPASMCs were divided into two groups:0(control group),0.5%CSE group.The mRNA and protein expression levels of transient receptor potential channel 1(TRPC1)and cyclin D1 in HPASMCs after CSE treatment were respectively detected by RT-PCR and Western blotting.The intracellular calcium ion concentration was measured by the calcium probe in each group.In the negative control group and TRPC1-siRNA transfection group,the proliferation of HPASMCs and the expression of cyclin D1 mRNA and protein were detected.Data were compared with one-way ANOVA(for multiple-group comparison)and independent t-test(for two-group comparison)followed by the least significant difference(LSD)test with the computer software SPSS 17.0.It was found that 0.5%and 1%CSE could promote the proliferation of HPASMCs(P<0.05),and the former was more effective than the latter(P<0.05),while 3%and above CSE had inhibitory effect on HPASMCs(P<0.05).The mRNA and protein expression levels of TRPC1 and cyclin D1 in 0.5%and 1%CSE groups were significantly higher than those in the control group(P<0.05),while those in 3%CSE group were significantly decreased(P<0.05).Moreover,the proliferation of HPASMCs and the expression of cyclin D1 mRNA and protein in TRPC1-siRNA transfection group were significantly reduced as compared with those in the negative control group(P<0.05).It was concluded that low concentration of CSE can promote the proliferation of HPASMCs,while high concentrations of CSE inhibit HPASMCs proliferation.These findings suggested that CSE induced proliferation of HPASMCs at least in part via TRPC1-mediated cyclin D1 expression.展开更多
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 ful...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.展开更多
BACKGROUND Endometrial stromal sarcoma(ESS)is a rare malignant mesenchymal tumor.Early in the disease,the findings on magnetic resonance imaging are similar to those of leiomyoma.When the lesion involves both vascular...BACKGROUND Endometrial stromal sarcoma(ESS)is a rare malignant mesenchymal tumor.Early in the disease,the findings on magnetic resonance imaging are similar to those of leiomyoma.When the lesion involves both vascular and cardiac tissue,it might be misdiagnosed as intravenous leiomyomatosis,which is not common in the clinic.CASE SUMMARY We present the case of a 34-year-old female patient with tumor embolus,which extended from the right iliac vein and ovarian vein to the inferior vena cava(IVC),and then to the right atrium and right ventricle,and finally protruded into the pulmonary artery.The patient had undergone a hystero-myomectomy 7 years previously.Based on the findings of the imaging examinations,the diagnosis of intravenous leiomyomatosis was considered preoperatively.The patient then underwent complete resection of the endovascular and intracardiac tumor embolus.The postoperative pathology results confirmed metastatic ESS with endovascular and intracardiac involvement.The patient was discharged from hospital in good condition,and there was no sign of recurrence 5 mo after the operation.CONCLUSION Extending from the iliac vein and ovarian vein to the IVC,this metastatic ESS invaded both vascular and cardiac tissues.For patients with ESS involving vascular and cardiac tissues,pathological examinations are essential for the differential diagnosis,such as intravenous leiomyomatosis.In addition,due to the high recurrence rate of ESS,long-term and close follow-up evaluation is necessary.展开更多
Acute myocardial infarction(AMI)with left ventricular(LV)dysfunction patients,the most common cause of cardiogenic shock(CS),have acutely deteriorating hemodynamic status.The frequent use of vasopressor and inotropic ...Acute myocardial infarction(AMI)with left ventricular(LV)dysfunction patients,the most common cause of cardiogenic shock(CS),have acutely deteriorating hemodynamic status.The frequent use of vasopressor and inotropic pharmacologic interventions along with mechanical circulatory support(MCS)in these patients necessitates invasive hemodynamic monitoring.After the pivotal Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness trial failed to show a significant improvement in clinical outcomes in shock patients managed with a pulmonary artery catheter(PAC),the use of PAC has become less popular in clinical practice.In this review,we summarize currently available literature to summarize the indications,clinical relevance,and recommendations for use of PAC in the setting of AMI-CS.展开更多
BACKGROUND Pulmonary artery-to-left atrial fistula is a variant of pulmonary arteriovenous fistula and is a developmental anomaly.Delayed presentation,cyanosis and effort intolerance are some of the important features...BACKGROUND Pulmonary artery-to-left atrial fistula is a variant of pulmonary arteriovenous fistula and is a developmental anomaly.Delayed presentation,cyanosis and effort intolerance are some of the important features.The diagnosis is confirmed by computed tomography or pulmonary artery angiography.Catheter-based closure is preferred to surgery.CASE SUMMARY Left pulmonary artery-to-left atrial fistula is rare.A 40-year-old male presented with effort intolerance,central cyanosis,and recurrent seizures.He had a large and highly tortuous left pulmonary artery-to-left atrial fistula associated with a large aneurysmal sac in the course.Catheter-based closure was performed using a vascular plug.CONCLUSION Left pulmonary artery-to-left atrial fistula is relatively uncommon compared to right pulmonary artery-to-left atrial fistula.Percutaneous closure by either a transeptal technique or guide wire insertion into the pulmonary vein through the pulmonary artery is preferred.The need for an arteriovenous loop depends on the tortuosity of the course of the fistula and the size of the device to be implanted because a larger device needs a larger sheath,necessitating firm guide wire support to facilitate negotiation of the stiff combination of the delivery sheath and dilator.展开更多
A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardi...A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.展开更多
Background Transthoracic Doppler echocardiography is recommended for screening the presence of pulmonary hypertension(PH).However,the accuracy of pulmonary artery systolic pressure(PASP) estimated by Doppler echocardi...Background Transthoracic Doppler echocardiography is recommended for screening the presence of pulmonary hypertension(PH).However,the accuracy of pulmonary artery systolic pressure(PASP) estimated by Doppler echocardiographic is still unknown.Methods We conducted a retrospective study on 102 patients with idiopathic pulmonary arterial hypertension who underwent Doppler echocar-diography within 72 hours before right heart catheterization. During this time,all patients were stable without any specific drug therapy.Results There was moderate correlation between Doppler echocardiographic and right heart catheteriza- tion measurements of PASP(r =0.642,P【0.001).Using Bland-Altman analytic methods,the bias for the echocardio-graphic estimates of PASP was 6.65 mm Hg with 95%limits of agreement ranging from -47.62 to 34.30 mm Hg.There were 58.8%cases with absolute differences over 10 mm Hg between the two methods.Overestimation and underestimation of PASP by Doppler echocardiography occurred in 15.7% (16/102) and 43.1%(44),respectively.The magnitude of pressure underestimation and overestimation was insignificant (24.52±12.15 vs.25.69±16.09,P=0.765),while the corresponding diagnostic categories of severity that each subject would fall into for each technique are not in good agreement. The diagnostic categories of 16 overestimated patients were in accordance.During 44 underestimated patients,20.5%of patients had their pressure underestimated within one diagnostic category(minor error);4.5%of the underestimates were with two diagnostic categories(major error).Conclusions Transthoracic Doppler echocardiography may frequently be inaccurate in estimating PASP and could not replace the right heart catheterization.展开更多
Objective To review the clinical experience of reconstruction of pulmonary artery(PA) by a patch of autologus pericardium or azygous venae for non-small cell lung cancer. Methods Between March 1992 and August 2009,62 ...Objective To review the clinical experience of reconstruction of pulmonary artery(PA) by a patch of autologus pericardium or azygous venae for non-small cell lung cancer. Methods Between March 1992 and August 2009,62 patients with locally advanced central lung cancer received sleeve resection and reconstruction of PA.展开更多
Objective Lung injury occurred during cardiopulmonary bypass as a result of both ischemic reperfusion injury and systemic inflammatory response is critical for patients’recovery. This study was designed to establish ...Objective Lung injury occurred during cardiopulmonary bypass as a result of both ischemic reperfusion injury and systemic inflammatory response is critical for patients’recovery. This study was designed to establish a convenient and appropriate mode for pulmonary arteryperfusion and evaluate its effects on the展开更多
Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data...Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data from medical records for 5 patients (4 boys and 1 girl,age from 1 to 18 years on admission) who had been treated in Wuhan Asia Heart Hospital between 2005 and 2009展开更多
文摘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.
文摘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.
基金Supported by the Natural Science Foundation of Shanxi Province,No.20210302123346Shanxi Provincial Health Commission“Four batch”Science and Technology Innovation Project of Medical Development,No.2021XM45.
文摘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.
基金Supported by Doctoral Research Foundation of The First Affiliated Hospital of Kunming Medical University,No.2022BS030and Scientific Research Foundation of Yunnan Education Department,No.2023Y0630.
文摘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.
文摘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.
基金supported by the National Natural Science Foundation of China(No.82000300).
文摘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.
基金supported by fund from Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences(2021-I2M-1–049)National High Level Hospital Clinical Research Funding(2022-NHLHCRF-LX-01-0203)
文摘Takayasu arteritis(TA)is a rare systemic vasculitis of the aorta and its primary branches,which usually occurs in young women.Due to its insidious onset and lack of specific symptoms,this disease can be easily misdiagnosed or missed.Approximately 50%of the patients having TA with pulmonary artery involvement develop pulmonary hypertension(PH).The 3-year survival rate among patients with TA-related PH is lower than that among patients with TA alone.Early balloon pulmonary angioplasty(BPA)can improve the clinical symptoms and survival of patients with stable TA.To the best of our knowledge,this is the first case reported in the English literature in which a“Guidezilla”catheter was used during BPA to treat stenosis and occlusion of the pulmonary artery caused by Takayasu arteritis(TA).
文摘BACKGROUND Uterine intravenous leiomyomatosis is defined as leiomyoma tissue invading the vein outside the leiomyoma.Reports of extension to the right pulmonary artery are relatively rare.CASE SUMMARY We describe a 31-year-old woman with a benign leiomyoma that extended into the right ventricular lumen,causing mechanical obstruction and corresponding clinical symptoms.Tumors located in the pulmonary artery can cause pulmonary artery obstruction.After diagnosis,surgical treatment should be performed as soon as possible.CONCLUSION In this case,the uterine leiomyoma extended to the right pulmonary system,which is clinically rare.
文摘BACKGROUND Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery,which has a low incidence rate and is easily misdiagnosed as pulmonary embolism.There is no standard protocol for the treatment of primary intimal sarcoma of the pulmonary artery.CASE SUMMARY This study reports a patient with primary intimal sarcoma of the pulmonary artery who was admitted to our hospital in 2017.The clinical characteristics,diagnosis,treatment and outcome of the patient were retrospectively analyzed.The patient was a Chinese Han male aged 44 years.He had three consecutive episodes of syncope,and was thus admitted to a local hospital.Computed tomography pulmonary angiography showed multiple lesions with abnormal densities in the pulmonary trunk,left pulmonary artery,mediastinum and pericardium,which were consistent with recurrence after tumor resection.He underwent surgery,and was pathologically diagnosed with intimal sarcoma of the pulmonary artery.He relapsed 3 mo after surgery,and apatinib was administered.His condition was stable after 4 mo,with tolerable and controllable adverse reactions.He subsequently died 19 mo after surgery.CONCLUSION Primary intimal sarcoma of the pulmonary artery has no specific clinical or imaging manifestations.The diagnosis of this disease depends on histopathology and immunohistochemistry,and has a poor clinical prognosis.Surgical treatment is currently a favorable option for primary intimal sarcoma of the pulmonary artery,and targeted therapy may provide new insights for the development of effective treatment methods.
文摘BACKGROUND Since 1923,only a few hundred cases of pulmonary arterial sarcoma(PAS)have been reported.It is easy for PAS to be misdiagnosed as pulmonary thromboembolism,which makes treatment difficult.The median survival time without surgical treatment for PAS is only 1.5-3 mo.Echocardiography is widely used in screening for pulmonary artery space-occupying lesions in patients with chest pain,dyspnea,and cough;furthermore,it is typically considered the first imaging examination for patients with PAS.CASE SUMMARY In May 2017,a 39-year-old male patient experienced chest pain with no particular obvious cause.At that time,the cause was thought to be pulmonary embolism.In July 2017,positron emission tomography–computed tomography revealed spaceoccupying lesions in the right lung and multiple metastases in both lungs.The lesions of the right lung were biopsied,and pathology revealed undifferentiated sarcoma.Chemotherapy had been performed since July 2017 in another hospital.In December 2019,the patient was admitted to our hospital for the sake of CyberKnife treatment.Echocardiography suggested:(1)A right ventricular outflow tract(RVOT)solid mass of the main pulmonary artery;and(2)mild pulmonary valve regurgitation.Ultrasonography showed the absence of a thrombus in the deep veins of either lower limb.CONCLUSION PAS is a single,central space-occupying lesion involving the RVOT and pulmonary valve.Echocardiography of PAS has its own characteristics.
文摘Cigarette smoking contributes to the development of pulmonary artery hypertension(PAH).As the basic pathological change of PAH,pulmonary vascular remodeling is considered to be related to the abnormal proliferation of pulmonary artery smooth muscle cells(PASMCs).However,the molecular mechanism underlying this process remains not exactly clear.The aim of this research was to study the molecular mechanism of PASMCs proliferation induced by smoking.Human PASMCs(HPASMCs)were divided into 6 groups:0%(control group),cigarette smoking extract(CSE)-treated groups at concentrations of 0.5%,1%,2%,5%,10%CSE respectively.HPASMCs proliferation was observed after 24 h.HPASMCs were divided into two groups:0(control group),0.5%CSE group.The mRNA and protein expression levels of transient receptor potential channel 1(TRPC1)and cyclin D1 in HPASMCs after CSE treatment were respectively detected by RT-PCR and Western blotting.The intracellular calcium ion concentration was measured by the calcium probe in each group.In the negative control group and TRPC1-siRNA transfection group,the proliferation of HPASMCs and the expression of cyclin D1 mRNA and protein were detected.Data were compared with one-way ANOVA(for multiple-group comparison)and independent t-test(for two-group comparison)followed by the least significant difference(LSD)test with the computer software SPSS 17.0.It was found that 0.5%and 1%CSE could promote the proliferation of HPASMCs(P<0.05),and the former was more effective than the latter(P<0.05),while 3%and above CSE had inhibitory effect on HPASMCs(P<0.05).The mRNA and protein expression levels of TRPC1 and cyclin D1 in 0.5%and 1%CSE groups were significantly higher than those in the control group(P<0.05),while those in 3%CSE group were significantly decreased(P<0.05).Moreover,the proliferation of HPASMCs and the expression of cyclin D1 mRNA and protein in TRPC1-siRNA transfection group were significantly reduced as compared with those in the negative control group(P<0.05).It was concluded that low concentration of CSE can promote the proliferation of HPASMCs,while high concentrations of CSE inhibit HPASMCs proliferation.These findings suggested that CSE induced proliferation of HPASMCs at least in part via TRPC1-mediated cyclin D1 expression.
文摘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.
文摘BACKGROUND Endometrial stromal sarcoma(ESS)is a rare malignant mesenchymal tumor.Early in the disease,the findings on magnetic resonance imaging are similar to those of leiomyoma.When the lesion involves both vascular and cardiac tissue,it might be misdiagnosed as intravenous leiomyomatosis,which is not common in the clinic.CASE SUMMARY We present the case of a 34-year-old female patient with tumor embolus,which extended from the right iliac vein and ovarian vein to the inferior vena cava(IVC),and then to the right atrium and right ventricle,and finally protruded into the pulmonary artery.The patient had undergone a hystero-myomectomy 7 years previously.Based on the findings of the imaging examinations,the diagnosis of intravenous leiomyomatosis was considered preoperatively.The patient then underwent complete resection of the endovascular and intracardiac tumor embolus.The postoperative pathology results confirmed metastatic ESS with endovascular and intracardiac involvement.The patient was discharged from hospital in good condition,and there was no sign of recurrence 5 mo after the operation.CONCLUSION Extending from the iliac vein and ovarian vein to the IVC,this metastatic ESS invaded both vascular and cardiac tissues.For patients with ESS involving vascular and cardiac tissues,pathological examinations are essential for the differential diagnosis,such as intravenous leiomyomatosis.In addition,due to the high recurrence rate of ESS,long-term and close follow-up evaluation is necessary.
文摘Acute myocardial infarction(AMI)with left ventricular(LV)dysfunction patients,the most common cause of cardiogenic shock(CS),have acutely deteriorating hemodynamic status.The frequent use of vasopressor and inotropic pharmacologic interventions along with mechanical circulatory support(MCS)in these patients necessitates invasive hemodynamic monitoring.After the pivotal Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness trial failed to show a significant improvement in clinical outcomes in shock patients managed with a pulmonary artery catheter(PAC),the use of PAC has become less popular in clinical practice.In this review,we summarize currently available literature to summarize the indications,clinical relevance,and recommendations for use of PAC in the setting of AMI-CS.
文摘BACKGROUND Pulmonary artery-to-left atrial fistula is a variant of pulmonary arteriovenous fistula and is a developmental anomaly.Delayed presentation,cyanosis and effort intolerance are some of the important features.The diagnosis is confirmed by computed tomography or pulmonary artery angiography.Catheter-based closure is preferred to surgery.CASE SUMMARY Left pulmonary artery-to-left atrial fistula is rare.A 40-year-old male presented with effort intolerance,central cyanosis,and recurrent seizures.He had a large and highly tortuous left pulmonary artery-to-left atrial fistula associated with a large aneurysmal sac in the course.Catheter-based closure was performed using a vascular plug.CONCLUSION Left pulmonary artery-to-left atrial fistula is relatively uncommon compared to right pulmonary artery-to-left atrial fistula.Percutaneous closure by either a transeptal technique or guide wire insertion into the pulmonary vein through the pulmonary artery is preferred.The need for an arteriovenous loop depends on the tortuosity of the course of the fistula and the size of the device to be implanted because a larger device needs a larger sheath,necessitating firm guide wire support to facilitate negotiation of the stiff combination of the delivery sheath and dilator.
基金The project was funded by the Natural Science Foundation of Chongqing Science and Technology Commission(cstc2019jcyj-msxmX0866).
文摘A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.
文摘Background Transthoracic Doppler echocardiography is recommended for screening the presence of pulmonary hypertension(PH).However,the accuracy of pulmonary artery systolic pressure(PASP) estimated by Doppler echocardiographic is still unknown.Methods We conducted a retrospective study on 102 patients with idiopathic pulmonary arterial hypertension who underwent Doppler echocar-diography within 72 hours before right heart catheterization. During this time,all patients were stable without any specific drug therapy.Results There was moderate correlation between Doppler echocardiographic and right heart catheteriza- tion measurements of PASP(r =0.642,P【0.001).Using Bland-Altman analytic methods,the bias for the echocardio-graphic estimates of PASP was 6.65 mm Hg with 95%limits of agreement ranging from -47.62 to 34.30 mm Hg.There were 58.8%cases with absolute differences over 10 mm Hg between the two methods.Overestimation and underestimation of PASP by Doppler echocardiography occurred in 15.7% (16/102) and 43.1%(44),respectively.The magnitude of pressure underestimation and overestimation was insignificant (24.52±12.15 vs.25.69±16.09,P=0.765),while the corresponding diagnostic categories of severity that each subject would fall into for each technique are not in good agreement. The diagnostic categories of 16 overestimated patients were in accordance.During 44 underestimated patients,20.5%of patients had their pressure underestimated within one diagnostic category(minor error);4.5%of the underestimates were with two diagnostic categories(major error).Conclusions Transthoracic Doppler echocardiography may frequently be inaccurate in estimating PASP and could not replace the right heart catheterization.
文摘Objective To review the clinical experience of reconstruction of pulmonary artery(PA) by a patch of autologus pericardium or azygous venae for non-small cell lung cancer. Methods Between March 1992 and August 2009,62 patients with locally advanced central lung cancer received sleeve resection and reconstruction of PA.
文摘Objective Lung injury occurred during cardiopulmonary bypass as a result of both ischemic reperfusion injury and systemic inflammatory response is critical for patients’recovery. This study was designed to establish a convenient and appropriate mode for pulmonary arteryperfusion and evaluate its effects on the
文摘Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data from medical records for 5 patients (4 boys and 1 girl,age from 1 to 18 years on admission) who had been treated in Wuhan Asia Heart Hospital between 2005 and 2009
