目的:探讨西格列汀对大鼠肺缺血再灌注损伤(LIRI)的作用.方法:通过夹闭左肺动脉30min后松开的方法,创建肺组织缺血再灌注损伤模型.设立空白对照组(Sham组),对照组(I/R组),实验组(sitagliptin组),将30只,SD大鼠,随机分组,每组10只,检测各组肺组织的湿干比(W/D);HE染色光镜下比较各组肺组织病理改变;测定丙二醛(MDA)含量和抗氧化酶(SOD、GSH-Px)活性;测定NF-κB蛋白含量.结果:与假手术组对比,缺血再灌注组和西格列汀组的肺组织W/D、MDA、NF-κB含量升高,SOD、GSH-Px活性降低,而且肺组织病理也有明显变化;与缺血再灌注组对比,西格列汀组的肺组织W/D、MDA、NF-κB含量较低,SOD、GSH-Px活性较高,肺组织病理改变更轻.结论:西格列汀可减轻肺组织缺血再灌注损伤病理改变,并可能通过抑制NF-κB蛋白表达,减轻炎症反应和氧化应激,从而减轻大鼠的肺缺血再灌注损伤.

随着对他汀类药物研究的不断深入,越来越多的证据表明,他汀类药物还具有抗炎、抗氧化、调节血管内皮功能以及影响细胞代谢等诸多作用,这些独立于降脂作用以外的其他作用即他汀类药物的多效性.现就几种常见呼吸系统疾病在他汀类药物多效性中的获益做一简要介绍.

目的 分析14例三尖瓣机械瓣置换术的临床效果和患者生存情况,以提高三尖瓣疾病手术疗效.方法 分析2010年1月至2019年5月期间于北京安贞医院心脏外科十病房接受三尖瓣位机械瓣置换外科手术的14例患者的临床资料,其中女性9例(64.3％),男性5例(35.7％),平均年龄(51.2±8.3)岁;术前心功能(NYHA分级):Ⅱ级2例,Ⅲ级10例,Ⅳ级2例;术前经胸超声心动图显示,风湿性病变(8例),发育不全(3例),Ebstein畸形(2例),法洛四联症术后(1例).患者均接受三尖瓣机械瓣置换术,并对术后患者住院期间及远期情况进行随访总结.结果 14例患者住院期间2例死亡(住院死亡率14.2％),1例术后并发急性肾功能衰竭,术后97 d出院.随访11例,随访率91.7％,平均随访时间(54.3±15.2)月;随访患者术后心功能(NYHA分级)Ⅰ级1例(9.1％),Ⅱ级8例(72.7％),Ⅲ级2例(18.2％);1例术后3个月发生三尖瓣瓣周漏,再次行三尖瓣生物瓣置换;其余患者随访期间无死亡及瓣膜相关心血管事件.结论 尽管三尖瓣机械瓣置换手术适应症要求严格,手术风险高,但手术方式可行,术后效果理想;对于无法行三尖瓣修复或修复效果不理想的患者,三尖瓣机械瓣置换是可行的.

目的:验证孟鲁司特钠(montelukast)对大鼠肺缺血再灌注损伤(LIRI)的预防作用.方法:将40大鼠随机分四组,每组10只:A组为假手术组、B组为LIRI模型组、C组为孟鲁司特钠低剂量组(ML组)、D组为孟鲁司特钠高剂量组(MH组).A、B组连续4d,每日接受2 mL/kg 0.9％氯化钠溶液灌胃,C、D:连续4d,分别给予3 mg/kg、30 mg/kg孟鲁司特钠灌胃,溶剂为0.9％氯化钠溶液2 mL/kg.第5日,A组在相同的时间内仅接受麻醉下气管切开机械通气、开胸翻动、依次关胸,不进行缺血再灌注处理;其余三组:采用左肺动脉夹闭法制备大鼠肺缺血再灌注模型.造模后2h断颈处死大鼠,结扎左肺门并切除左肺.测定肺组织干湿重比(W/D),HE染色观察肺组织病理变化;测定丙二醛(MDA)含量、髓过氧化物酶(MPO)活性、超氧化物岐化酶活以及核因子-κB (NF-κB)含量.结果:与假手术组对比,肺组织W/D、MDA、NF-κB含量及MPO、SOD活性,以及肺组织病理改变在LIRI模型组、ML组和MH组均有明显变化,差异有统计学意义(P＜0.05);且ML组和MH组间的差异也具有统计学意义,且MH组病理改变更轻.结论:孟鲁司特钠可下调NF-κB蛋白表达,抑制炎性反应和氧化应激,来减轻大鼠肺缺血再灌注造成的肺损伤,且高浓度(30 mg/kg)较低浓度(10 mg/kg)保护作用更显著.

Background: The pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) is largely unknown. Proteomics offers an approach to overview the molecular activities and signal transduction pathways involved in specific disease processes. Objectives: In this study, the expression of proteins in endarterectomized tissues from patients with CTEPH was investigated in a novel strategy to explore the pathophysiology of this disease. Methods: We used the iTRAQ (isobaric tag for relative and absolute quantitation) approach combined with a Thermo Scientific Q Exactive MS analysis to compare the protein profiles in endarterectomized tissues from CTEPH patients and that of the control samples (mixture of cultured human pulmonary artery endothelial cells, human pulmonary artery smooth muscle cells, and human pulmonary fibroblasts). GO and KEGG analyses were performed to understand the functional classification and molecular activities of all the tissue-specific proteins, and the involved signal transduction pathways. Results: Six hundred and seventy-nine tissue-specific proteins were detected. Bioinformatic analysis showed that the major biological processes involving these proteins were: response to wounding, defense response, acute inflammatory response, immune response, complement activation, and blood coagulation. The main pathways involved were: complement and coagulation cascade, systemic lupus erythematosus, extracellular matrix-receptor interaction, cell adhesion molecules, FcεRI signaling, and leukocyte transendothelial migration. Conclusions: The present study revealed that immune and defense response might play an important role in CTEPH.

OBJECTIVE:To improve our understanding and facilitate early diagnosis of pulmonary artery sarcoma (PAS).PATIENTS AND METHODS:The clinical and radiological features of 9 histopathologically confirmed patients with PAS were retrospectively analyzed.RESULTS:Our PAS cohort consisted of 5 males and 4 females, with insidious presentation, the main symptoms were dyspnea (88.9%), syncope (33.3%), palpitations (22.2%), chest pain (22.2%), hemoptysis (11.1%) and constitutional symptoms, including fever (44.4%), fatigue (55.6%) and weight loss (55.6%). Elevated serum inflammatory markers, including an elevated erythrocyte sedimentation rate, lactate dehydrogenase and C-reactive protein, were recorded in 9 (100%), 7 (77.8%) and 8 (88.9%) patients, respectively. Computed tomography pulmonary angiography (CTPA) indicated that 8 (88.9%) patients had intraluminal defects, appearing as rounded, bulging or lobulated masses that display uneven signal intensification in contract-enhanced scans in the pulmonary arteries (PAs). Three (33.3%) of the patients had metastases outside of the PA. Before surgery, 7 patients had been misdiagnosed as pulmonary thromboembolic diseases (PTD), 2 as others, with mean misdiagnosis duration of 7.2 ± 7.1 months. All patients had received thrombolytic and/or anticoagulation therapy and their diseases progressed until receiving pulmonary endarterectomy.CONCLUSIONS:The clinical features of PAS that may help to differentiate it from PTD are its insidious onset, constitutional symptoms, increased serum inflammatory marker levels, lack of predisposing factors and unresponsiveness to thrombolytic and/or anticoagulation therapy. Typical CTPA features are crucial for suspicion of PAS. Early biopsy or surgery should be performed to confirm the diagnosis of PAS and improve its prognosis.

Objective: The operability evaluation for pulmonary thromboendarterectomy (PEA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) is always difficult. In this study, we aimed to identify a better indicator of operability in patients with surgically accessible CTEPH lesions. Methods: Two-hundred-eight patients with surgically accessible CTEPH lesions who underwent PEA at Beijing Anzhen Hospital from March 2001 to February 2014 were retrospectively reviewed. The occluded pulmonary segments (OPS) were assessed by ventilation/perfusion scintigraphy, pulmonary vascular resistance (PVR) was measured by right heart catheterization and the PVR/OPS ratio was calculated. Results: Seven (3.37%) early deaths occurred in the post-PEA period, six late deaths occurred during the mean follow-up period of 58.3 ± 39.7 months; the 5 year actuarial survival rate was 95.1% ± 3.5%. The PVR/OPS ratios of early and late death after PEA were significantly higher than those of early and late survival, respectively. A PVR/OPS ratio of <100 dyne·s·cm-5/OPS had much better specificity (88.7% vs. 69.2%) and sensitivity (92.3% vs. 38.5%) than did PVR alone in the prediction of early and late survival. The difference between the two areas under their receiver operating characteristic curves reached statistical significance (z test: Z=1.9917, P=0.046). Conclusion: The PVR/OPS ratio is a better indicator of operability for surgically accessible CTEPH than is PVR alone. Patients with a PVR/OPS ratio of <100 dyne·s·cm-5/OPS have better early and long-term outcomes after PEA.

目的 比较交叉连锁褥式缝合法与间断褥式缝合法修补巨型室间隔缺损术后的效果及残余漏发生率.方法 回顾性分析2011年2月至2013年4月我院41例巨型室间隔缺损采用交叉连锁褥式缝合法修补术患者的资料.将这41例患者作为交连组,平均年龄(18.7±12.3)岁,男女比例为31∶10.按交连组患者相同年龄、室间隔缺损大小及部位、肺动脉压及肺循环阻力共5项指标进行1比1匹配,选择安贞医院心外科同期以间断褥式缝合法修补的巨型室间隔缺损患者41例作为对照组,平均年龄(17.4±11.8)岁,男女比例为31∶10,进行回顾性配对对照分析.结果 全组无手术死亡,无围术期新发房室传导阻滞发生.围术期经超声检查发现对照组残余漏发生率为31.7％(13/41),交连组为0％(0/41),两组差异有统计学意义(x2=13.164,P=0.000).两组患者平均随访(18.2±6.1)个月,随访期内均无死亡,无新房室传导阻滞发生.术后1年两组在纽约心脏病学会心功能分级、经超声心动图检测肺动脉压的差异均无统计学意义.术后12个月复查超声心动图,对照组残余漏发生率为26.8％(11/41),交连组为0％ (0/41),两组差异有统计学意义(x2=10.499,P=0.001).结论 间断褥式缝合法修补合并中、重度肺动脉高压的巨型室间隔缺损术后的残余漏发生率较高,而采用交叉连锁褥式缝合法可有效减少残余漏的发生率.

目的:探讨肺动脉高压临床教学效果的方法.方法:肺动脉高压是现代临床医学中重要的疾病范畴,是危重病症.在临床教学中,存在着医疗风险高,而学员的医疗风险防范意识薄弱,基础也相对较薄弱,对肺动脉高压的临床内容理解不容易深入的难点.结果和结论:针对上述教学难点,采取分层化教学管理、理论与案例教学相结合、制定详细教学计划、紧密结合临床实际及重视基础理论教学等对策,可取得较好的教学效果.

Pulmonary endarterectomy is the gold standard treatment for chronic thromboembolic pulmonary hypertension and is potentially curative, although some patients are unsuitable for pulmonary endarterectomy and require alternative management. Lack of standardized assessment of pulmonary endarterectomy eligibility risks suboptimal treatment in some patients. We discuss the implications for future clinical trials and practice of a unique operability assessment in patients who have chronic thromboembolic pulmonary hypertension and were initially screened for inclusion in the CHEST-1 (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase Stimulator Trial-1) study. The CHEST-1 study evaluated riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent/recurrent pulmonary hypertension after pulmonary endarterectomy. Screened patients who were initially considered "inoperable'' underwent central independent adjudication by a committee of experienced surgeons, or local adjudication in collaboration with an experienced surgeon. Operability decisions were based on accessibility of thrombi and the association between pulmonary vascular resistance (PVR) and the extent of obstruction, using pulmonary angiography/computed tomography with ventilation/perfusion scintigraphy as the minimum diagnostic tests. Of 446 patients screened for CHEST-1, a total of 188 and 124 underwent central and local adjudication, respectively, after being initially considered to be "inoperable.'' After a second assessment by an experienced surgeon, 69 of these 312 "inoperable'' patients were deemed operable. Rigorous measures in CHEST-1 guaranteed that only technically inoperable patients, or patients who had persistent/recurrent pulmonary hypertension, were enrolled, thus ensuring that only patients for whom surgery was not an option were enrolled. This study design sets new standards for future clinical trials and practice in CTEPH, helping to ensure that patients who have CTEPH receive optimal treatment.

Objective Our aim was to investigate the effect and prognosis of surgical treatment to pulmonary artery sarcoma(PAS).Methods From November,2001 to January,2014,nineteen PAS were diagnosed,and 14 of them were surgically treated at Beijing Anzhen Hospital,and the data were retrospectively reviewed.Results All 19 patients underwent pulmonary artery CTA scan,and all showed a filling defect within the lumen of the pulmonary artery with a sign of wall eclipsing on pulmonary artery CTA; 14 patients were confirmed pulmonary artery sarcoma through histo-pathological investigation after surgery; the other five patients were confirmed to have FDG abnormal high intake mass shadow in PET-CT scan.Fourteen patients underwent surgical treatment,including 12 cases of pulmonary endarterectomy,two cases of pneumonectomy,and the other five patients had no indication for operation and died very soon.There were no perioperative deaths for surgically treated patients.Five patients without surgical treatment survived 3 days to one month after discharge.14 patients survived 5-46 months after surgical treatment,with an average survival of(16.8 ± 3.8) months,the difference between the two groups reached statistically significance(t test,P =0.0001).Of the 12 patients with pulmonary endarterectomy,the first four cases did not and the later eight cases did undergo deep hypothermic circulatory arrest during the procedure,and their average survival were(9.7 ± 2.8) and (18.3 ± 3.5) months respectively,and the difference between them reached a statistical significance.Of the 14 patients with surgical resection,9 patients did not and the other 5 did received adjuvant radiotherapy and chemotherapy,their average survival were(12.3 ±3.2) and(22.8 ± 13.3) months respectively,and the difference between the two groups reached statistically significance(t test,P =0.0387).Conclusion Radical surgical resection can provide the longer survival than non-surgical treatment to the PAS patients.Intraoperative deep hypothermic circulatory arrest helps to fully eliminate the tunor body,tumor thrombus and superimposed blood clots,thus helps to prolong the survival time.And adjuvant chemotherapy may further extend their survival.

Objective: Operation on the infrarenal aorta could cause ischemic-reperfusion (IR) injury in local tissues and remote organs (e.g. the lung). We aim to explore the method of reducing lung ischemia-reperfusion damage after lower limb IR with post conditioning (LIPC). Methods: Bilateral lower limb ischemia was performed in Sprague-Dawley (SD) rats, and then animals were divided into 4 groups: IR-Sham-operated, IR, post conditioned-IR (LIPC) and bilateral lower limb ischemia (LIR). The serum free radical, histological changes, Wet/Dry (W/D) ratio, levels of TNF-alpha, IL-6, cytokines and chemokines were tested and compared. Results: Post-conditioning could ameliorate histological injuries in the lung when compared to IR group. The serum free radical is significantly lower in LIPC group than IR groups. W/D ratio in LIPC groups is significantly lower. LIPC also could reduce the expression of cytokines and chemokines. Conclusion: post conditioning could reduce long-term damages of the lung after lower limb ischemic-reperfusion injury.

目的:比较分析各种诊断方法对肺动脉肉瘤(pulmonary artery sarcoma,PAS)的诊断正确率,以探讨提高肺动脉肉瘤的诊断正确率的方法.方法:回顾性分析2001年11月至2014年1月,安贞医院收治19例肺动脉肉瘤的CT平扫、超声心动图、胸部X线平片、心电图、通气灌注扫描及肺动脉CT造影(PACTA)对肺动脉肉瘤的诊断正确率和特点.结果:14例手术后病理学检查证实为肺动脉肉瘤;5例未行手术治疗者,正电子发射断层显像(PET-CT)检查显示为:氟脱氧葡萄糖摄取异常增高团块影.19例患者的PACTA均显示蚀壁征;对于前12例PAS,因为不认识肺动脉CTA“蚀壁征”而导致全部术前误诊;而后7例,因为发现PACTA“蚀壁征”均诊断正确.PACTA“蚀壁征”诊断正确率高于CT平扫、超声心动图、胸部X线平片、心电图及通气灌注扫描.结论:CT平扫、超声心动图、胸部平片、心电图及通气灌注扫描对肺动脉肉瘤的诊断均无特异性,均对PAS的鉴别诊断价值不大,而在PACTA中的“蚀壁征”征象具有肺动脉肉瘤疾病特征性,有助于肺动脉肉瘤与肺动脉栓塞性疾病的早期鉴别诊断.

OBJECTIVE:To investigate the clinical characteristics of pulmonary artery sarcoma (PAS) and to improve doctors' awareness and the early diagnosis of this disease.METHODS:The clinical data of 9 cases confirmed by operation and pathology of PAS from November 2001 to November 2012 in Beijing Anzhen Hospital were reviewed. The clinical characteristics, laboratory tests, imaging manifestation, pathology as well as follow-up were studied. Survival was determined by the Kaplan-Merier method.RESULTS:(1)Main clinical manifestations were chest distress (8/9), palpitation (2/9), syncope (2/9), cough (2/9), weight loss (2/9) and chest pain (1/9). (2)Lactate dehydrogenase (LDH) , high-sensitivity C-reactive protein (hs-CRP), erythrocyte sedimentation rate (ESR) were all elevated to different extents, but D-Dimer remained in the normal range. (3) Seven cases received CTPA examination and evidenced filling defect in the main pulmonary artery trunk (6/7), left pulmonary artery (6/7), right pulmonary artery (7/7). 7 cases received TTE examination and showed enlarged right ventricle (6/7) : mean right ventricular end-diastolic diameter was (38.54 ± 16.30) mm; enlarged right atrium (7/7): mean right atrium diameter was (55.11 ± 5.45) mm; and tricuspid insufficiency (7/7) : estimated mean pulmonary artery pressure was (81.14 ± 21.17) mmHg (1 mmHg = 0.133 kPa) , and pulmonary stenosis (5/7) . Four cases received deep venous ultrasound examination and deep venous thrombosis (DVT) was found in 1 patient. Four cases received Ganz catheter examination and the preoperative and postoperative mean pulmonary artery pressure was (52.00 ± 5.23) mmHg and (23.00 ± 5.60) mmHg, respectively. (4) All 9 patients received surgery and intimal sarcoma was diagnosed in all of them. Leiomyosarcoma was diagnosed in 3 cases, leiomyo-angiosarcoma was diagnosed in 1 case, undifferentiated sarcomas was diagnosed in 1 case. (5)The 1-, 3- and 5-month survival was 71.4%, 53.6% and 35.7%, respectively, median survival time was 5 months post surgery.CONCLUSION:PAS is difficult to differentiate with PTE. PAS should be suspected in patient with right atrium, right ventricular enlargement and early appeared right heart failure, and normal D-Dimer level. Outcome for PAS patients is poor despite surgery in this cohort.

目的:探讨成人冠状动脉异常起源于肺动脉的临床特点、诊断方法、治疗及意义.方法:北京安贞医院自2004年1月至2012年12月,共收治12例成人冠状动脉起源异常患者,对其临床资料、治疗情况及随访结果进行回顾性分析.结果:全组男性2例,女性10例,年龄16～66岁,平均年龄为(39.2±17.9)岁.其中10例为左冠状动脉起源于肺动脉,2例为右冠状动脉起源于肺动脉.患者临床表现缺乏特异性,在确诊前均误诊为其他疾病.全组均经冠状动脉造影,心脏超声心动图(UCG),冠状动脉CTA等检查而确定诊断.其中9例患者行外科手术重建冠状动脉,2例患者拒绝手术,1例因严重心功能不全而失去手术时机.手术患者中2例患者因合并重度二尖瓣反流而同时行二尖瓣置换术,1例患者因合并冠心病同时行冠状动脉旁路移植术.无围手术期死亡病例及术后并发症.随访6 ～48个月,平均随访(27±13)个月,患者术后均症状消失或明显缓解,一般体力活动不受限.UCG提示:术后左心室舒张末径从[(47.4±7.2) mm减小到(41.8±6.5)mm,P＜0.01],左心室的短轴缩短率(FS)从[(24.3±11.3)％增至(36.1±11.6)％,P＜0.01)].未手术治疗患者1例死于心力衰竭.1例失访,1例仍时有活动后气短、心慌等症状.结论:成人冠状动脉异常起源于肺动脉无特征性临床表现,除了冠状动脉造影,UCG和冠状动脉CT等无创检查对确定诊断具有重要意义.手术治疗重建双冠状动脉系统是目前首选的治疗方案,可以改善心脏血供,从而防止猝死、心功能不全等严重心脏事件的潜在危险.

Background We carried out a retrospective data review of patients with systemic to pulmonary shunts that underwent surgical repair between February 1990 and February 2012 in order to assess preoperative pulmonary vascular dynamic risk factors for predicting early and late deaths due presumably to pulmonary vascular disease. Methods and Results A total of 1024 cases of congenital systemic-to-pulmonary shunt and advanced pulmonary vascular disease beyond infancy and early childhood were closed surgically. The mean follow up duration was 8.5±5.5 (range 0.7 to 20) years. Sixty-one in-hospital deaths (5.96%, 61/1024) occurred after the shunt closure procedure and there were 46 late deaths, yielding 107 total deaths. We analyzed preoperative pulmonary vascular resistance index (PVRI), pulmonary vascular resistance index on pure oxygen challenge (PVRIO), difference between PVRI and PVRIO (PVRID), Qp∶Qs, and Rp∶Rs as individual risk predictors. The results showed that these individual factors all predicted in-hospital death and total death with PVRIO showing better performance than other risk factors. A multivariable Cox regression model was built,and suggested that PVRID and Qp∶Qs were informative factors for predicting survival time from late death and closure of congenital septal defects was safe with a PVRIO<10.3 WU.m2 and PVRID>7.3 WU.m2 on 100% oxygen. Conclusions All 4 variables, PVRI, PVRIO, PVRID and Qp∶Qs, should be considered in deciding surgical closure of congenital septal defects and a PVRIO<10.3 WU.m2 and PVRID>7.3 WU.m2 on 100% oxygen are associated with a favorable risk benefit profile for the procedure.

目的:分析肺动脉肉瘤(pulmonary artery sarcoma,PAS)患者的临床及CT影像学表现,以提高对本病的认识.方法:回顾性分析自2001年11月至2012年11月,北京安贞医院收治并经病理证实的PAS病例共9例,对其临床特点、实验室检查及影像学表现进行讨论.结果:9例患者确诊年龄32～ 67岁,中位数年龄为43岁.临床表现主要为胸闷、心悸、胸痛及晕厥等,2例表现为低氧血症.7例患者术前诊断为中央型肺动脉栓塞并行溶栓治疗无效.7例D-二聚体正常,6例乳酸脱氢酶(LDH)升高.9例患者均行CT肺动脉成像,8例表现为肺动脉腔内充盈缺损,为分叶状、向外隆起、边缘清晰;1例表现为沿主肺动脉及右肺动脉管壁浸润性生长;单纯累及右肺动脉1例,累及左、右肺动脉及分支部2例,累及主肺动脉及左右肺动脉主干6例,其中2例导致右肺动脉闭塞,9例均显示不同程度强化,3例同时发现肺动脉外转移灶.结论:PAS临床表现及实验室检查缺乏特异性,CT肺血管成像对本病的诊断具有重要意义.当临床发现有类似肺动脉血栓栓塞症状,D-二聚体正常,溶栓治疗无效时,应警惕本病可能.特别是CTPA发现病灶边缘饱满、隆起,甚至有分叶,增强扫描明显不均匀强化者,高度提示本病.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease characterized by misguided thrombolysis and remodeling of pulmonary arteries. MicroRNAs are small non-coding RNAs involved in multiple cell processes and functions. During CTEPH, circulating microRNA profile endued with characteristics of diseased cells could be identified as a biomarker, and might help in recognition of pathogenesis. Thus, in this study, we compared the differentially expressed microRNAs in plasma of CTEPH patients and healthy controls and investigated their potential functions. Microarray was used to identify microRNA expression profile and qRT-PCR for validation. The targets of differentially expressed microRNAs were identified in silico, and the Gene Ontology database and Kyoto Encyclopedia of Genes and Genomes pathway database were used for functional investigation of target gene profile. Targets of let-7b were validated by fluorescence reporter assay. Protein expression of target genes was determined by ELISA or western blotting. Cell migration was evaluated by wound healing assay. The results showed that 1) thirty five microRNAs were differentially expressed in CTEPH patients, among which, a signature of 17 microRNAs, which was shown to be related to the disease pathogenesis by in silico analysis, gave diagnostic efficacy of both sensitivity and specificity >0.9. 2) Let-7b, one of the down-regulated anti-oncogenic microRNAs in the signature, was validated to decrease to about 0.25 fold in CTEPH patients. 3) ET-1 and TGFBR1 were direct targets of let-7b. Altering let-7b level influenced ET-1 and TGFBR1 expression in pulmonary arterial endothelial cells (PAECs) as well as the migration of PAECs and pulmonary arterial smooth muscle cells (PASMCs). These results suggested that CTEPH patients had aberrant microRNA signature which might provide some clue for pathogenesis study and biomarker screening. Reduced let-7b might be involved in the pathogenesis of CTEPH by affecting ET-1 expression and the function of PAECs and PASMCs.

OBJECTIVE:To characterize the differential diagnostic characteristics and the surgical treatment efficacy of pulmonary artery sarcoma (PAS).METHODS:From November 2001 to January 2014, 19 PAS patients were diagnosed and 14 of them underwent surgery at Beijing Anzhen Hospital. And their data were retrospectively reviewed.RESULTS:All 19 patients underwent pulmonary artery computed tomography angiography (CTA) scan. All showed a filling defect within the lumen of pulmonary artery with a sign of wall eclipsing. And 14 of them had pulmonary artery sarcoma confirmed through postoperative histopathological examination while another 5 patients were confirmed to have FDG abnormal high intake mass shadow on Positron emission tomography-computed tomography (PET-CT) scan. Fourteen patients underwent surgery, including pulmonary endarterectomy (n = 12) and pneumonectomy (n = 2), and another five had no indication for operation and died shortly. No perioperative death occurred for surgical patients. Five non-surgical patients survived (20.3 ± 11.2) days after discharge. And 14 postoperative patients survived (16.8 ± 3.8) months. The difference between two groups reached statistical significance (P = 0.000). The survival difference between two surgical procedures and between two pathological classifications did not reach statistical significance. Nine patients did not while another 5 received adjuvant radiotherapy and chemotherapy. Their average survivals were (12.3 ± 3.2) and (22.8 ± 4.3) months respectively. And the inter-group difference reached statistical significance (P = 0.000).CONCLUSIONS:The sign of wall eclipsing on pulmonary artery CTA scan is pathognomonic for PAS. Radical surgical resection provides a longer survival than non-surgery and adjuvant chemotherapy may further extend survival.