CT肺动脉造影测定脊柱室间隔角评价慢性血栓栓塞性肺动脉高压肺血管阻力的价值探讨

目的以肺动脉造影及右心导管为标准,采用多层螺旋计算机断层扫描肺动脉造影（CTPA）测定脊柱室间隔角,分析脊柱室间隔角与血流动力学的关系,探讨脊柱室间隔角在评价慢性血栓栓塞性肺动脉高压（CTEPH）血流动力学的价值。方法回顾性纳入2006年1月至2010年6月确诊CTEPH的患者89例（男57例,女32例）,年龄（53.08±12.43）岁,并随机选取最终经临床证实无肺栓塞及肺动脉高压的89例患者为对照组。所有CETPH患者均于右心导管检查前1～2 d内行CTPA检查。2名放射科医师共同根据CTPA图像测定CT肺动脉栓塞指数（采用Qanadli肺栓塞指数和Mastora肺栓塞指数）及其脊柱室间隔夹角。结果 CTEPH患者脊柱室间隔夹角为65.13°±12.26°,对照组脊柱室间隔夹角为39.69°±5.84°,两组存在显著差异（t=14.479,P=0.000）。CTPEH患者Qanadli栓塞指数为（42.50±17.67）%,Mastora栓塞指数为（30.02±15.53）%。脊柱室间隔夹角与Qanadi栓塞指数（r=0.094,P=0.552）及Mastora栓塞指数无显著相关性（r=0.025,P=0.873）。Spearman相关分析显示脊柱室间隔夹角与肺血管阻力呈中度正相关（r=0.529,P=0.000）,与右房压呈弱正相关（r=0.270,P=0.010）,与末梢血氧饱和度、肺动脉收缩压、舒张压及平均肺动脉压、肺毛细血管嵌压无显著相关性（P〉0.05）。ROC曲线显示脊柱室间隔角为67.55°时,对估测肺血管阻力（PVR）≥1000 dyne.s.cm-5的诊断灵敏度为0.714,特异度为0.778,ROC曲线下面积为0.764。结论脊柱室间隔夹角能够较好地反映CTEPH患者的PVR变化,对评价CTPEH具有重要的参考价值。     

肺动脉血栓内膜剥脱术治疗慢性栓塞性肺动脉高压的临床研究

目的 探讨肺动脉内膜剥脱术在治疗慢性栓塞性肺动脉高压的效果、诊治经验及手术指证.方法 5例慢性栓塞性肺动脉高压的患者,均在体外循环下行肺动脉切开血栓取出术＋肺动脉内膜剥脱术.结果 本组病人手术过程顺利,无手术死亡,无术后残余肺动脉高压、灌注性肺水肿等并发症,术后病人肺动脉压力和肺血管阻力明显降低,复查心脏彩超均示肺动脉血流通畅,血管管壁光滑,术后顺利出院,术后随访6～12个月,效果满意.结论 肺动脉切开血栓取出术＋肺动脉内膜剥脱术对于慢性栓塞性肺动脉高压的患者,尤其是内科溶栓术无效的患者,有良好的治疗效果.     

Riociguat

肺高压是一种进行性、致死性疾病，其特征是肺动脉压升高，导致血管重构、右心肥大和心衰。研究表明，肺高压与内源性血管扩张剂NO的产生受损有关。Riociguat是首个新一类可溶性鸟苷酸环化酶（sGC）激动剂，它直接刺激sGC，增强其对低水平NO的敏感度。目前应用本品对肺动脉高压（PAH）患者进行III期临床、对慢性血栓栓塞性肺高压（CTEPH）患者进行II期临床研究，表明疗效好，易耐受，不良反应轻微。     

Journey of a patient with chronic thromboembolic pulmonary hypertension

Right ventricle (RV) dysfunction is a key outcome determinant and a leading cause of death for patients with chronic thromboembolic pulmonary hypertension (CTEPH). In this report, we followed the 5-year clinical journey of a patient with CTEPH. The tricuspid pressure gradient was significantly increased in the early phase of CTEPH and “normalized” at the late phase of this patient’s clinical journey, but this “normalized” gradient is not a positive treatment response but rather an ominous sign of advancing right heart failure owing to an exhaustion of RV contractile function. Thus, appropriate interpretation of the tricuspid pressure gradient change is of importance for assessing RV dysfunction and treatment outcome during follow-up in patients with CTEPH. Besides systolic pulmonary artery pressure (SPAP), other RV functional parameters such as tricuspid annular plane systolic excursion, RV fractional area change, and RV longitudinal strain, together with clinical markers, may provide additional guidance regarding functional improvement or progression in patients with CTEPH.     

慢性血栓栓塞性肺动脉高压的流行病学研究

目的调查西京医院呼吸与危重症医学科确诊肺栓塞患者中慢性血栓栓塞性肺动脉高压(CTEPH)的发病率及诊疗状况,为完善我国CTEPH的临床流行病学资料提供依据。方法采用回顾性研究的方法,调查2008年1月至2012年5月西京医院呼吸与危重症医学科确诊肺栓塞患者中CTEPH的发病率及诊疗状况。结果 CTEPH的发病率为5.24%。62.90%的静脉血栓栓塞/肺栓塞(VTE/PE)患者危险因素不详,53.85%的CTEPH患者危险因素不详。CTEPH中38%无VTE病史,62%无明显的急性肺动脉栓塞事件。所有CTEPH患者均未行肺动脉内膜剥脱术(PTE)治疗,53.85%的患者仅给予抗凝治疗。结论西京医院呼吸与危重症医学科发现的CTEPH的发病率较高,与临床医生对CTEPH的危险因素认识不足,对肺栓塞的溶栓、抗凝治疗不够充分有关,故急需提高临床医生对CTEPH的规范化诊疗水平。     

多层螺旋CT在肺动脉高压诊断中的应用

目的探讨多层螺旋CT在肺动脉高压诊断中的应用价值。方法回顾性分析了22例肺动脉高压患者的临床及多层螺旋CT资料,其中慢性血栓栓塞性肺动脉高压9例,原发性肺动脉高压7例,肝肺综合征2例,结缔组织病2例,嗜酸性细胞增多-肌痛综合征1例,纤维性纵隔炎1例。在CT上观察肺实质变化,如磨玻璃样改变、“马赛克”征、小叶间隔增厚等,及纵隔、肺血管、右室扩大和室间隔移位等。结果CT肺动脉造影诊断了95%的肺动脉高压及右心功能不全,9例显示为肺动脉血栓,慢性血栓栓塞性肺动脉高压的“马赛克”征发生率明显高于原发性肺动脉高压(P=0.034)。结论CT肺动脉造影可准确诊断肺动脉高压,并为病因诊断提供有效信息。     

靶向药物治疗慢性血栓栓塞性肺动脉高压疗效评价

目的：观察肺动脉高压靶向药物治疗慢性血栓栓塞性肺动脉高压是否可以改善患者运动耐量、心功能及血流动力学指标。方法收集首都医科大学附属北京世纪坛医院2009年3月-2012年3月就诊的慢性血栓栓塞性肺动脉高压患者25例,单独或联合给予口服内皮素受体拮抗剂、5型磷酸二酯酶抑制剂（西地那非或伐地那非）或吸入前列环素类药物（伊洛前列素）治疗。16周后复查患者6 min步行距离、心功能和超声心动图评价疗效。结果治疗16周后患者6 min步行距离由基线的（321±118） m增加至（351±102） m,平均增加30 m （P＜0.0001）;超声心动图提示平均肺动脉压力（mean pulmonary artery pressure,mPAP）由（53.96±9.23） mmHg （1 mmHg=0.133 kPa）降至（46.36±10.95） mmHg （P＜0.0001）,三尖瓣环收缩期位移（tricuspid annular plane systolic excursion,TAPSE）由（1.3±0.4） cm增加至（1.4±0.3） cm （P＜0.05）,心肌做功指数（Tei指数）由（0.71±0.19）降至（0.64±0.19）,氨基末端脑钠肽前体（N-terminal pro-brain natriuretic peptide,NT-proBNP）水平由（1942.24±880.24） pg/ml降至（861.22±378.31） pg/m （P＜0.0001）。结论肺动脉高压靶向药物治疗慢性血栓栓塞性肺高血压有效,可改善患者16周的运动耐量和血流动力学指标。     

V/Q显像与CT肺动脉造影在慢性血栓栓塞性肺动脉高压不同栓塞水平中的研究

目的对比肺通气/灌注显像(ventilation/perfusion scan,V/Q)与CT肺动脉造影(CT pulmonary angiography,CTPA)在慢性血栓栓塞性肺动脉高压(chronic thromboembolic pulmonary hypertension,CTEPH)肺段动脉及亚段动脉栓子的检出效能。方法选取经临床Wells评分、临床资料、实验室检查及X线肺动脉造影综合确诊为慢性血栓栓塞性肺动脉高压的39例患者,其中男28例,女11例,平均年龄(51.0±16.1)岁,合并近期手术者4例、心力衰竭者7例、既往存在急性肺栓塞12例、合并下肢深静脉血栓26例。临床Wells评分,高度可能性者17例,中度可能性者22例。所有患者均接受V/Q单光子发射计算机断层成像术(single photon emission CT,SPECT)断层显像、CTPA及X线肺动脉造影(pulmonary artery angiography,PAG)检查,检查于3～7 d内完成并由放射科及核医学科有经验医师进行独立、双盲法阅片。采用SPSS 13.0统计软件对V/Q SPECT及CTPA与PAG段及亚段的检查结果进行χ2检验及Kappa一致性检验。结果对于肺段动脉栓子,V/Q SPECT与CTPA的敏感度、特异度、准确度分别为84.7%、77.7%,94.6%、98.2%,90.9%、91.2%;亚段肺动脉栓子,V/Q SPECT与CTPA敏感度、特异度、准确度分别为63.2%、50.4%,94.8%、94.8%,93.8%、83.9%。结论 V/Q显像对段及亚段病变定位的敏感性较高,临床中V/Q显像正常即可排除CTEPH,若存在异常须进一步行CTPA、PAG及磁共振成像(magnetic resonance imaging,MRI)等检查进一步明确。     

体外膜肺氧合在肺动脉血栓内膜剥脱术围术期的应用

目的探讨体外膜肺氧合(ECMO)在慢性血栓栓塞性肺动脉高压(CTEPH)患者行血栓内膜剥脱术(PTE)中的应用。方法回顾性分析安贞医院自2008年9月至2014年1月,因PTE术后不能顺利脱离体外循环(CPB)或出现PTE术后相关并发症而应用ECMO辅助治疗的7例患者,均采用静脉-动脉ECMO模式进行支持,以患者生存和死亡结果将其分为两组。回顾分析这些患者的临床资料,以及辅助原因,辅助相关并发症等。结果 7例患者中成功脱机6例,存活4例,死亡3例。结论对PTE术后伴有严重并发症或PTE术后脱机困难的危重症患者,应用ECMO治疗有一定的临床价值。     

Pulmonary oligemia maneuver can alleviate pulmonary artery injury during pulmonary thromboendarterectomy procedure

Background Pulmonary thromboendarterectomy (PTE) has evolved as a treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to characterize if pulmonary oligemia maneuver (POM) can alleviate pulmonary artery injury during PTE procedure.

Methods A total of 112 cases of CTEPH admitted to Beijing Anzhen Hospital from March 2002 to August 2011 received PTE procedure. They were retrospectively classified as non-POM group (group A, n=55) or POM group (group B, n=57). Members from group B received POM during rewarming period, whereas members from group A did not.

Results There were three (5.45%) early deaths in group A, no death in group B (0) (Fisher’s exact test, P=0.118). Six patients in group A needed extracorporeal membrane oxygenation (ECMO) as life support after the PTE procedure, no patients in group B needed ECMO (Fisher’s exact test, P=0.013). The patients in group B had a shorter intubation and ICU stay, lower mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR), higher partial pressure of oxygen in artery (PaO₂) and arterial oxygen saturation (SaO₂) and less medical expenditure than patients in group A. With a mean follow-up time of (58.3 ± 30.6) months, two patients in group A and one patient in group B died. The difference of the actuarial survival after the procedure between the two groups did not reach statistical significance. Three months post the PTE procedure, the difference of residual occluded pulmonary segment between the two groups did not reach statistical significance (P=0.393).

Conclusion POM can alleviate pulmonary artery injury, shorten ICU stay and intubation time, and lower down the rate of ECMO after PTE procedure.

     

Imaging in pulmonary hypertension, part 2: Large vessel diseases

Pulmonary hypertension is defined by physiological parameters but there are numerous causes that differ in their pathogenesis, management and prognosis. Causes include chronic cardiac or pulmonary diseases and diffuse small vessel disease but also a range of large vessel obstructive diseases. The physiological manifestation of all these diseases is increased pulmonary vascular resistance and pulmonary arterial hypertension, and while clinical features may provide a clue to diagnosis, imaging plays a fundamental role in establishing a precise diagnosis and therefore guiding therapy. Chronic thromboembolic pulmonary hypertension (CTEPH) is the most common large vessel cause of pulmonary hypertension. It is increasingly recognised as a major cause of morbidity and mortality which is underdiagnosed and often diagnosed late. The importance of CTEPH is that for patients in whom the distribution of disease lies predominantly in the proximal vasculature there is potential for symptomatic and physiological cure by surgical pulmonary endarterectomy. More distal disease may be suitable for medical management. Increased awareness on behalf of both clinicians and imagers is therefore paramount. However, there are other rare causes or large vessel obstruction/stenosis such as large vessel vasculitis, pulmonary artery tumour, fibrosing mediastinitis, congenital stenosis or extrinsic compression of the pulmonary arteries/veins. Atypical imaging appearance such as unilateral central pulmonary artery obstruction should lead to consideration of a diagnosis other than CTEPH.     

Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: preliminary exploration in China

Background Pulmonary endarterectomy is safe and effective surgical treatment for chronic thromboembolic pulmonary hypertension. This study aimed to evaluate the efficacy of pulmonary endarterectomy in treatment of thromboembolic pulmonary hypertension. Methods A retrospective study of 15 patients who underwent pulmonary endarterectomy in Beijing Chaoyang Hospital was performed. Obvious pulmonary hypertension and hypoxemia were observed in all patients. Bilateral pulmonary endarterectomy was performed under cardiopulmonary bypass with profound hypothermic circulatory arrest. Results Two patients （2/15） died of residual postoperative pulmonary hypertension and bleeding complication. The other 13 cases had significant decrease in systolic pulmonary artery pressure （（92.8＋_27.4） mmHg vs. （49.3＋18.6） mmHg） and pulmonary vascular resistance （（938.7±464.1） dynes.s.cm5 vs. （316.8±153.3） dynes＇s.cm5）, great improvement in cardiac index （（2.31：LK）.69） L.min-l.m2 vs. （3.85±1.21） L.min-l.m2）, arterial oxygen saturation （0.67±O.11 vs. 0.96±0.22） and mixed venous 02 saturation （0.52±0.12 vs. 0.74±0.16） postoperatively compared to preoperative data. Mid-term follow-up showed that the cardiac function of all cases returned to NYHA class I or II, with great improvement in 6-minute walking distance （（138±36） m） and quality of life. Conclusions Bilateral pulmonary endarterectomy using cardiopulmonary bypass with the aid of deep hypothermia and circulatory arrest can effectively reduce pulmonary hypertension and provide good mid-term hemodynamic and symptomatic results with low surgical mortality rate and few complications.     

V/Q显像与SPECT-CT断层显像在慢性血栓栓塞性肺动脉高压中的研究

目的对比肺通气/灌注平面显像与肺灌注SPECT-CT同机融合断层显像在慢性血栓栓塞性肺动脉高压肺段动脉及亚段动脉栓子的检出效能。方法选取经临床Wells评分、临床资料、实验室检查及X线肺动脉造影综合确诊为慢性血栓栓塞性肺动脉高压的35例患者,其中男25例,女10例,平均年龄（52.4±16.5）岁,合并近期手术者3例、心力衰竭者7例、既往存在急性肺栓塞10例、合并下肢深静脉血栓21例;临床Wells评分,高度可能性者15例,中度可能性者20例。所有患者均接受肺通气/灌注平面显像、肺灌注SPECT-CT断层同机融合显像及X线肺动脉造影检查,检查于5 d内完成并由核医学科及放射科有经验医师进行独立、双盲法阅片。采用SPSS19.0统计软件对肺通气/灌注平面显像、SPECT-CT同机融合显像及X线肺动脉造影段及亚段的检查结果进行χ2检验及Kappa一致性检验。结果对于肺段动脉栓子,肺通气/灌注平面显像与肺灌注SPECT-CT的敏感度、特异度、准确度分别为89.63%、69.41%、77.14%;92.53%、93.57%、93.17%。亚段肺动脉栓子,肺通气/灌注平面显像与肺灌注SPECT-CT敏感度、特异度、准确度分别为60.97%、89.05%、82.14%;63.87%、94.0%、86.59%。结论肺灌注SPECT-CT在保留了肺通气/灌注平面显像对肺段及亚段病变定位较高的敏感性同时,提高了对肺段及亚段病变定位的特异性。     

电子束CT在慢性栓塞性肺动脉高压诊断与外科治疗中的应用

目的:探讨电子束CT(EBCT)检查在慢性栓塞性肺动脉高压(CTPH)诊断及外科治疗前后的应用价值。方法:2003年12月~2006年5月,我院共有8例经临床病史、实验室检查及EBCT检查确诊的CTPH患者成功接受了肺动脉血栓内膜剥脱术(PTE),并于术后1~2周内接受了EBCT复查,对其手术前后EBCT的影像学表现及相应临床指标的对应关系进行了综合分析。结果:术前EBCT检查8例患者中主肺动脉或左右肺动脉干内显示附壁充盈缺损共7支,肺叶动脉及肺段动脉显示部分充盈缺损共28支、缺支共6支;2例患者于肺窗可见"马赛克征",3例可见条索影;CT测量主肺动脉直径平均为36.5mm。PTE术后复查EBCT显示所有患者肺动脉及分支内充盈缺损均有不同程度减少、消失,其中左右肺动脉及主肺动脉内附壁充盈缺损均消失,血栓清除率达100%,22支肺叶及肺段动脉充盈缺损影消失,6支完全闭塞的肺血管分支开通,血栓清除率82.4%(28/34),CT测量主肺动脉直径平均为34.2mm,较术前回缩。8例患者临床症状的缓解程度与EBCT血栓征象消除状况有良好的一致性。结论:EBCT对CTPH的诊断和治疗有重要指导价值,也是PTE手术治疗后随访的可靠方法。     

慢性栓塞性肺动脉高压外科治疗连续62例无死亡临床分析

目的 分析应用肺动脉血栓内膜剥脱术治疗慢性栓塞肺动脉高压的效果及诊治经验.方法 回顾性分析2002年10月至2008年9月于北京安贞医院手术治疗62例慢性栓塞性肺动脉高压患者的临床资料,按临床病理分型分为中央型组(46例)、外周型组(16例).全组均在深低温停循环下行肺动脉血栓内膜剥脱术.结果 围术期全组无死亡,围术期残余肺动脉高压15例,肺再灌注损伤23例,后者均经延长辅助呼吸支持治疗及体外膜肺治疗治愈.单因素分析显示外周型CTEPH是术后残余肺动脉高压的危险因子,中央型CTEPH是术后肺再灌注损伤的危险因子(P＜0.05).62例围术期生存患者术后72 h Swan-Ganz导管及血气指标较术前显著改善,肺动脉收缩压从(91 ±38) mm Hg 降至(53±21) mm Hg, 而肺循环阻力从(916±548) dyn·s·cm-5降至(368±302) dyn·s·cm-5(t=6.896,P=0.0001), 动脉血氧分压从 (51±7) mm Hg 升至(90±7) mm Hg, 动脉血氧饱和度从87.0%±3.9% 升至96.1%±3.3%,P＜0.01.随访期间无死亡.46例术后复查核素血流灌注通气扫描结果 显示,原灌注缺损部位恢复显像,或(及)肺动脉CT造影提示原不显影区充盈造影剂.2例患者于术后2年、3年再发双下肢静脉血栓,但因有下腔静脉滤网而未发肺动脉栓塞,全组术后3年免除再次栓塞率为96.7%±2.8%.发生出血并发症3例.全组抗凝相关出血线性发生率为2.47%患者年,再发下肢静脉栓塞线性发生率为1.64%患者年.术后长期生存62例患者中,心功能NYHA分级Ⅰ级38例、Ⅱ级20例、Ⅲ级2例、Ⅳ组2例.结论 肺动脉血栓内膜剥脱术治疗慢性肺动脉栓塞术后有较好围术期及中长期生存率,口服华法令抗凝相关的再发肺动脉栓塞、下肢静脉栓塞率、出血并发症线性发生率均在可接受范围.     

The effects and mechanism of ginsenoside Rg1 on myocardial remodeling in an animal model of chronic thromboembolic pulmonary hypertension

Background

Recent studies haveshown that ginsenoside Rg1, extracted from the dry roots of Panax notoginseng as a traditional Asian medicine, plays an anti-fibrosis role in myocardial remodeling. However, the mechanism still remains unclear. In the present study, we investigate the effect of ginsenoside Rg1on the collagenic remodeling of myocardium in chronic thromboembolic pulmonary hypertension (CTEPH), and its potential mechanism.

Methods

A rat model of CTEPH was established by injecting thrombi through the jugular vein wice in2 weeks. Four weeks later, four groups (Group A: normal rats + normal saline; Group B: normal rats + Rg1; Group C: CTEPH model + normal saline; Group D: CTEPH model + Rg1) were established. Normal saline and Rg1 were administrated by intraperitoneal injection. Ineach group, we measured the hemodynamic parameters, as well as the right ventricle to left ventricle (RV/LV) thickness ratio. Myocardial tissue sections of the RV were stained by hematoxylin-eosin +gentian violet and the morphological characteristics were observed by light microscopy. The matrix metalloproteinases (MMP) -2 and −9 were detected by the western blot.

Results

Compared with Group A and Group B, the right ventricular systolic pressure was significantly increased in Group C and significantly decreased in Group D. Compared with Group A and Group B, the RV/LV thickness ratio of the rats was significantly higher in Group C and Group D. There was significant fibrosis with collagen in Group C compared with Group A and Group B, and less significant changes in Group D were observed compared with those in Group C. The expression of MMP-2 and MMP-9 exhibited a significant decrease in Group C and was also significantly decreased in Group D compared withGroup A and Group B. Also, a negative linear relationship was shown between collagen-I and the expression of MMP-2 and MMP-9.

Conclusions

Our animal study showed that ginsenoside Rg1 positively affects myocardial remodeling and pulmonary hemodynamics in CTEPH. Upregulation of the expression of MMP-2 and MMP-9 could explain the beneficial effects of ginsenoside Rg1 in CTEPH.     

Current practice for pulmonary hypertension

Objective To investigate the current practice of pulmonary hypertension including current epidemiology, diagnosis and treatment. Data sources The review was based on data obtained from the published articles and guidelines. Study selection Articles with high level of evidence or current best evidence in each issue were selected to be reviewed. Results Overall prevalence of pulmonary hypertension was 0.3% to 6% with left heart disease occupying the most proportion, followed by pulmonary disease, pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. In diagnosis, a flow diagram of diagnosis of pulmonary hypertension, differential diagnosis of pulmonary hypertension and how to determine the severity of pulmonary hypertension are explained including recent development of magnetic resonance imaging and gene abnormality study on bone morphogenetic protein receptor ll. In treatment, newly- developed pulmonary vasodilators and the way to use them are shown to treat pulmonary hypertension. Conclusion Safer and more effective treatment algorithm and basic researches and clinical trials are warranted to be explored.     

吸入伊洛前列素对慢性血栓栓塞性肺动脉高压患者血液动力学和氧动力学的即刻影响

目的 评价吸入伊洛前列素对慢性血栓栓塞性肺动脉高压(CTEPH)患者血液动力学和氧动力学的即刻影响.方法 采用前瞻性研究方法.对18例确诊的CTEPH患者置入右心导管,测定基线和吸入伊洛前列素20μg后即刻血液动力学和氧动力学参数并进行比较.结果 吸入伊洛前列素后,CTEPH患者肺动脉平均压从(53±11)下降到(47±14)mm Hg(1 mm Hg=0.133 kPa,P<0.01),右心房压从(10±6)下降到(7±6)mm Hg(P<0.01),肺血管阻力从(821±194)下降到(681±199)dyn·s·cm-5 (P<0.01),而心率、平均血压、肺动脉楔压、心排血量和体循环血管阻力无明显变化(均P>0.05);动脉血氧分压(PaO2)从(58±11)下降到(52±6)mm Hg(P<0.01),并伴有肺内分流和肺泡气-动脉血氧分压差增加[(27±11)%比(33±9)%,(86±39)比(93±38)mm Hg,均P<0.01].结论 吸入伊洛前列素可即刻改善CTEPH患者的血液动力学,但同时可增加肺内分流,导致PaO2下降.     

THE ALTERATION OF THE PULMONARY ARTERY FLOW SPECTRUM WITH PULMONARY HYPERTENSION

Objeaive and method. To research the alteration of pulmonary flow spectrum of patients with congeni-tal heart disease and the relation between pulmonary flow spectrum and pulmonary artery pressure, 33 pa-tients with congenital heart disease were examined by echocardicgraphy and catheterization. Results. The result showed that the spectrum peak early occurring in patients with pulmonary hyper-tension and the degree of the spectrum peak early occurring was related to ptflmovary artery pressure posi-tively. The result suggested that when the pulmovary artery systolic pressure was over 100 mmHg, the flow spectrum showed slender pattern usually. Conclusion. The slender pattern in pulmonary flow spectrum could be a semi-quantity parameter to predict that the pulmonary artery systolic pressure more than 100 mmHg.     

慢性血栓栓塞性肺动脉高压患者肺血管平滑肌细胞膜电位的改变

目的探讨分离培养的慢性血栓栓塞性肺动脉高压患者肺血管平滑肌细胞的方法,观察其电生理学特性。方法分离慢性血栓栓塞性肺动脉高压(chronic thromboembolic pulmonary hypertension,CTEPH)患者内膜剥脱术后的肺组织标本(CTEPH患者组)和肺癌或肺大泡患者正常肺组织标本(正常对照组)的肺血管平滑肌细胞,并进行体外培养,用特异性抗体(smoothmuscle-α-actin,SM-α-actin)进行免疫荧光鉴定。膜片钳记录两组肺血管平滑肌细胞的静息膜电位和动作电位,分析比较两者的异同。结果①酶解法成功分离肺血管平滑肌细胞,经鉴定SM-α-actin阳性细胞达90%以上;②CTEPH患者组肺血管平滑肌细胞静息膜电位(-21.05 mV±2.20 mV,n=11)明显低于正常对照组(-38.12 mV±2.28 mV,n=10),细胞膜电位降低了约45%,处于明显去极化状态(P<0.001);③CTEPH患者组肺血管平滑肌细胞动作电位时程(action potential duration,APD):APD50(0.185 s±0.035 s),APD75(0.277 s±0.053 s),APD90(0.333 s±0.064 s)与正常对照组APD50(0.100 s±0.016 s),APD75(0.150 s±0.024 s),APD90(0.180 s±0.028 s)相比,均明显延长(P<0.05)。结论 CTEPH患者肺血管平滑肌细胞静息膜电位明显减小,动作电位时程延长,提示CTEPH患者肺血管平滑肌细胞存在明显的电生理学特性改变,该变化可能是细胞膜上电压依赖性钙离子通道激活、钙离子浓度增加、肺血管重构发生的重要因素。