慢性肺血栓栓塞症与慢性血栓栓塞性肺动脉高压

慢性肺血栓栓塞症（简称慢性肺栓塞）与慢性血栓栓塞性肺动脉高压（CTEPH）被认为是急性肺栓塞的远期并 发症。近年来,随着对慢性血栓认识的深入以及介入、手术等治疗技术的发展,慢性肺栓塞和CTEPH患者的诊治进 展迅速,患者预后显著改善。但另一方面,慢性肺栓塞和CTEPH的诊治相对复杂,而国内医师对其认识相对不足,临 床不规范诊治的情况并不少见。文章重点阐述慢性肺栓塞和CTEPH的基本概念及诊治思路。     

核素显像诊断肺动脉血栓栓塞及疗效评价

<正> 肺动脉血栓栓塞(简称肺栓塞)是内源性或外源性栓子堵塞肺动脉或其分支导致肺循环障碍的一种临床和病理生理综合征,若发生肺出血或坏死则称为肺梗死.肺栓塞是一种常见病,在美国每年约有70余万患者,其中20%发生死亡,占死因的第三位,仅次于肿瘤和心肌梗死.在我国,目前尚无确切的流行病学调查资料,但根据北京阜外医院900余例心血管病尸检资料证实,有肺段以上较大肺栓塞者100例,占11%,占风湿性心脏病尸检的29%,心肌病的26%,肺源性心脏病的19%,说明肺栓塞并发于心血管疾病者已相当多见.但是,肺栓塞血栓的主要来源是盆腔和下肢深静脉的炎症和血栓,约占70%以上,因此,探测下肢深静脉病变十分重要.     

核素肺灌注显像对肺栓塞患者诊断的临床研究

目的 研究放射性核素肺灌注显像在肺动脉栓塞中的价值.方法 对临床怀疑有肺动脉栓塞的21例患者进行了放射性核素肺灌注显像.结果 21例患者中有13例患者出现不同程度的多发肺段血流灌注异常,其中19个肺段出现放射性缺损区,48个肺段出现放射性稀疏区.结论 放射性核素肺灌注显像是诊断肺动脉栓塞一种无创伤性的有效的检查方法.     

肺灌注/通气显像在肺动脉栓塞诊断中的价值

目的:评价核素肺通气/灌注(V/Q)显像在肺动脉栓塞(PE)诊断中的价值。方法:回顾总结40例核素肺灌注/通气显像与肺动脉造影(CPA)诊断PE的对照研究。结果:31例患者V/Q显像示不相匹配的肺叶、肺段或多发亚肺段的放射性分布稀疏或缺损,提示为PE,其中肺动脉造影证实30例为PE,1例多发大动脉炎;9例患者的V/Q显像为相匹配的肺叶、肺段或多发亚肺段的稀疏或缺损,提示不是PE,与肺动脉造影结果一致。核素V/Q显像诊断PE的灵敏度为100%(30/30例),特异性为90.0%(9/10例),准确性为97.5%(39/40例)。结论:多数PE患者通过核素肺V/Q显像可以作出明确诊断,少数肺V/Q显像与临床表现不符的患者需行肺动脉造影。     

慢性血栓栓塞性肺动脉高压的临床研究进展

慢性血栓栓塞性肺动脉高压是一种较为常见的疾病,虽然目前认为慢性血栓栓塞性肺动脉高压是急性肺栓塞的一种临床结局,但部分慢性血栓栓塞性肺动脉高压患者并无明确肺栓塞病史.由于临床医师对此疾病的认识尚不够深入,故往往是在对呼吸困难患者进行超声心动图检查时才怀疑此疾病.慢性血栓栓塞性肺动脉高压的确诊依赖于通气/灌注肺扫描和肺动脉造影.本文对慢性血栓栓塞性肺动脉高压的发病机制、危险因素和临床表现以及诊断研究进展进行综述.     

慢性血栓栓塞性肺动脉高压的诊断和治疗体会

目的提高对慢性血栓栓塞性肺动脉高压(CTEPH)的认识和早期诊治水平。方法对我院1994年10月至2009年9月收治的104例CTEPH患者的临床资料进行回顾性分析。结果 CTEPH患者中男性多见,男:女为1.97:1,平均年龄46.4±16.7岁,病史37.0±19.6月。临床主要表现有活动性呼吸困难/气促、胸闷、胸痛及咯血等。所有患者超声心动图均示肺动脉高压,其他表现有右心室扩大、三尖瓣返流、主肺动脉内占位性病变等。而血管超声示下肢静脉血栓形成占68.3%。患者确诊均通过肺动脉造影(PPA,CTPA或MRPA)。入院时54例被误诊,误诊率为52%。41例给予内科治疗,63例行肺动脉血栓内膜剥脱术,死亡6例,总手术死亡率9.5%。57例成功手术者,肺动脉收缩压由术前93.2±35.8mmHg降至术后的51.2±14.1mmHg,动脉血氧分压由52.8±7.9mmHg升至86.4±9.2mmHg,二组前后相比有显著性差异(P0.05)。结论 CTEPH临床上比较少见,容易被误诊。UCG和CTPA有较大诊断价值。内科治疗效果欠佳,肺动脉血栓内膜剥脱术应作为首选治疗手段。     

肺动脉血栓内膜剥脱术治疗慢性栓塞性肺动脉高压效果分析

目的分析慢性栓塞性肺动脉高压(CTEPH)患者应用肺动脉血栓内膜剥脱术(PTE)治疗的围手术期及中、晚期效果。方法选择CTEPH患者81例,按临床病理分型分为中央型组60例和外周型组21例,在深低温停循环下行PTE,随访5年,观察生存率。回顾性按年龄将患者分为老年组(≥60岁,19例)和非老年组(<60岁,62例),比较2组早期及晚期生存率。结果中央型组围手术期死亡1例。外周型组患者体外循环时间、主动脉阻断时间、深低温停循环时间均显著长于中央型组;围手术期肺动脉高压危象发生率显著高于中央型组,肺再灌注损伤发生率显著低于中央型组;肺动脉收缩压、肺循环阻力显著高于中央型组(P<0.05,P<0.01)。随访期间1例死亡,5年生存率为98.77%。老年组与非老年组术后早期及晚期生存率差异无统计学意义(P>0.05)。结论 PTE治疗CTEPH患者有较好的围手术期及中、晚期生存率,老年患者术后早期及晚期生存率与非老年患者无显著差异。术前准确评估,可提高PTE早期及中、晚期生存率。     

慢性血栓栓塞性肺动脉高压的诊治进展

慢性血栓栓塞性肺动脉高压(CTEPH)属于肺源性高血压中的一种特殊类型,由于临床缺乏特异性表现,CTEPH的诊断存在一定的挑战性。尽管CTEPH患者的预后较差,但是CTEPH是一种可能完全治愈的肺高血压。目前CTEPH主要疗法包括肺动脉血栓内膜剥脱术、介入治疗、药物治疗等。本文就CTEPH的诊疗进展作一综述。     

肺血栓栓塞52例临床分析

目的提高对肺血栓栓塞（ＰＴＥ）的认识。方法经肺核素显像、肺动脉造影和Ｘ线胸片等方法，对５２例ＰＴＥ进行回顾性分析。结果近年ＰＴＥ发病率似有升高趋势。引发深静脉血栓性静脉炎是导致ＰＴＥ的危险因素，除手术、下肢创伤（２６９％）、慢性心、肺疾病（２３１％）和恶性肿瘤（１５４％）外，肾病综合征占１９２％；肺灌注显像呈ＰＴＥ低度可疑（ＬＰ）者中，１３３％（２／１５）被诊断为ＰＴＥ；如按其中临床上ＰＴＥ高度可疑者计算，则４０％（２／５）为ＰＴＥ。本组病死率为１９２％，未治疗者为７７８％，治疗者为７０％。结论ＰＴＥ仍是一个被人们认识不足的疾病，因此应给予重视     

重视慢性血栓栓塞性肺动脉高压的诊治和研究

慢性血栓栓塞性肺动脉高压(CTEPH)的治疗技术进展迅速。随着手术、介入、靶向药物的开发以及综合治疗策略的观念更新,CTEPH患者的预后已有显著改善。但CTEPH的诊断和治疗仍面临很大挑战,如何正确应用影像学检查方法早期识别和诊断CTEPH？如何根据不同患者的表型选择个体化治疗方案？如何以研究推动CTEPH患者的规范化...     

Pilot study comparing SPECT perfusion scintigraphy with CT pulmonary angiography in chronic thromboembolic pulmonary hypertension

Background and objective: The management of chronic thromboembolic pulmonary hypertension (CTEPH) is dependent on the extent of pulmonary artery obstruction, which is usually evaluated by planar perfusion scanning and CT pulmonary angiography (CTPA). We previously reported that SPECT perfusion scanning is more sensitive than planar scanning for detecting vascular obstruction in CTEPH. The purpose of this study is to compare SPECT with CTPA for detecting segmental pulmonary artery obstruction in CTEPH. Methods: SPECT and CTPA were carried out before pulmonary endarterectomy in 12 CTEPH patients. Field experts documented the anatomical distribution of perfusion defects disclosed by SPECT, the anatomical distribution of pulmonary arterial filling defects disclosed by CTPA and the segmental anatomy of the vascular obstructions based on a review of clinical and pathology records, without knowledge of scan results. Results: Clinical/pathological evaluation disclosed 140 obstructed (15.5 ± 2.5 per patient) and 40 unobstructed lung segments. SPECT scanning identified 87/140 (62%) of the obstructed and 29/40 (72%) of the unobstructed segments. By comparison, CTPA identified 67/140 (47.8%) of the obstructed and 32/40 (80%) of the unobstructed segments. Sensitivity for detecting obstructed segments was significantly higher for SPECT compared with CTPA (62 ± 4.1% vs 47.8 ± 2.9%, respectively; P = 0.03). Conclusions: SPECT is more sensitive than CTPA for identifying obstructed segments in this small sample of CTEPH patients. However, even SPECT under‐represents the extent of vascular obstruction from this disease.     

慢性栓塞性肺动脉高压大鼠肺动脉重塑与尾加压素Ⅱ及其受体的表达

目的 研究尾加压素Ⅱ(U Ⅱ)蛋白和mRNA及U Ⅱ受体(UT)mRNA在慢性栓塞性肺动脉高压大鼠肺动脉的表达,探讨其在病程中的作用.方法 雄性Wistar大鼠,麻醉后经颈静脉注入体外制备的血栓栓子,2周后同法进行2次栓塞,造模成功大鼠分为2周组、4周组、8周组、12周组,全程腹腔注射抗纤维溶解剂氨甲环酸,达到目标日期后行以下检查:(1)测量平均肺动脉压(mPAP);(2)用免疫学检验和原位杂交的方法检测不同节段肺动脉U Ⅱ蛋白和mRNA表达及UT mRNA表达;(3)在光镜下观察肺动脉显微结构的变化,测定肺动脉相对中膜厚度(PAMT)和管壁面积/管总面积(WA/TA).采用SPSS 13.0软件,所有数据以-x±s表示,组间比较采用单因素方差分析,组间差异采用LSD方差分析.结果 (1)栓塞后4、8及12周组的大鼠mPAP值分别为(19.9±6.2)mm Hg(1 mm Hg=0.133 kPa)、(23.8±4.1)mm Hg、(27.4±5.4)mm Hg,较对照组明显升高(F值为13.75,P＜0.01),PAMT百分比分别为42.6±11.16、47.82±10.02、53.79±10.41,WA/TA百分比分别为22.75±6.79、25.32±4.90、27.05±7.71,较对照组均明显增大(F值分别为5.52和6.61,P均＜0.01);(2)栓塞后肺动脉U Ⅱ蛋白和U Ⅱ mRNA以及UT mRNA表达上调,细小动脉较中型动脉变化更为明显,4、8及12周组肺细小动脉U Ⅱ mRNA和UT mRNA及U Ⅱ蛋白平均吸光度值分别为0.138±0.019、0.144±0.022、0.173±0.021和0.126±0.028、0.146±0.029、0.157±0.025,与对照组相比明显升高(F值分别为30.39、30.78和14.49,P均＜0.01),随着栓塞时间的延长,表达呈明显增加的趋势;(3)肺细小动脉U Ⅱ蛋白和mRNA及UT mRNA的平均吸光度值均与mPAP、PAMT呈正相关关系(r值分别为0.822、0.866、0.846;0.675、0.712、0.756,P均＜0.01).结论 慢性栓塞性肺动脉高压大鼠出现明显肺动脉重构,尾加压素Ⅱ蛋白和mRNA及其UT mRNA在肺动脉表达明显上调,其动态变化与肺动脉高压、肺血管重构的病理过程明显相关.     

Recent progress in the diagnosis and management of chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension caused by non-resolving thromboembolisms of the pulmonary arteries. In Japan, in contrast to Western countries, CTEPH is more prevalent in women. A Japanese multicenter study reported that a form of CTEPH unrelated to deep vein thrombosis is associated with HLA-B?5201, suggesting that this form of CTEPH may be associated with vasculopathy. CTEPH can be cured by pulmonary endarterectomy, provided that the thrombi are surgically accessible; thus, early diagnosis is important, and all patients with exertional dyspnea should be evaluated for pulmonary hypertension. Ventilation/perfusion scans provide an excellent non-invasive means to distinguish CTEPH from pulmonary arterial hypertension. Similarly, computed tomographic pulmonary angiograms allow for the detection of thrombi and evaluation of pulmonary hemodynamics in a minimally invasive manner. Importantly, the absence of subpleural perfusion on pulmonary angiograms can suggest the presence of small vessel disease. Small vessel disease might be involved in the pathogenesis of CTEPH, and its detection is essential in preventing operative death. Although no modern therapies for pulmonary arterial hypertension have been approved for treatment of CTEPH, a recent randomized control trial of riociguat in patients with CTEPH demonstrated that riociguat significantly improved 6-min walking distance. Further investigations into treatments that target endothelial dysfunction and hyperproliferative CTEPH cells are needed. Recently, balloon pulmonary angioplasty has emerged as a promising treatment modality in Japan. A specialized medical team, including at least one expert surgeon, should make decisions regarding patients' candidacy for pulmonary endarterectomy and/or balloon pulmonary angioplasty.     

Update on chronic thromboembolic pulmonary hypertension

Chronic, unresolved thromboemboli are an important cause of pulmonary hypertension (PH) with specific treatment strategies differing from other types of PH. Chronic thromboembolic pulmonary hypertension (CTEPH) is classified as group 4 PH by the World Health Organization. It is a rare, but underdiagnosed, complication of acute pulmonary embolism that does not resolve and results in occlusion of large pulmonary arteries with a fibro-thrombotic material. The etiology of CTEPH remains uncertain, and it is unknown why certain patients with acute pulmonary embolism develop this disorder. The evaluation for CTEPH is an important part of the evaluation for PH in general, and it is crucial not to overlook this diagnosis, as it is the only form of PH that is potentially curable. Patients diagnosed with CTEPH should be referred to an expert center for consideration of pulmonary endarterectomy, and surgical removal of the chronic thromboembolic material. Not all patients with CTEPH are surgical candidates, however, and there are emerging treatments—medical therapy and balloon pulmonary angioplasty—that have shown benefit in this patient population. Without treatment, CTEPH can lead to progressive pulmonary vascular obstruction, right heart failure, and death. Thus, it is important for clinicians to recognize this subtype of PH. In this review, we provide an overview of current understanding of the pathogenesis of CTEPH and highlight recommendations and recent advances in the evaluation and treatment of CTEPH.     

Non-invasive methods in the diagnosis of chronic major-vessel thromboembolic pulmonary hypertension

The diagnosis of chronic major-vessel thromboembolic pulmonaryhypertension was made in a 63-year-old man by non- invasivemethods (Echo, CT, MRI) and confirmed by pulmonary angiographyand surgery. All these methods proved very useful in detectingand defining this rare entity.     

经皮腔内肺动脉成形术治疗慢性血栓栓塞性肺动脉高压的有效性及安全性评估

目的评估经皮腔内肺动脉成形术(PTPA)治疗慢性血栓栓塞性肺动脉高压(CTEPH)患者的有效性和安全性。方法该研究为前瞻性单臂试验。纳入2017年1月至2019年6月武汉亚洲心脏病医院心外科确诊的CTEPH患者。以明确诊断CTEPH的时间为基线, 收集入选患者的基线临床资料, 包括年龄、性别、世界卫生组织(WHO)功能分级、6 min步行距离、N末端B型利钠肽原(NT-proBNP)水平以及右心导管测定的血流动力学指标等。患者分次行PTPA, 统计每位患者扩张血管数, 术后24周随访并复查右心导管。记录手术安全性指标, 包括全因死亡、围术期并发症、再灌注肺水肿等。结果共入选患者19例, 年龄(56.3±12.5)岁, 男性7例。入选患者分别进行了1～7次PTPA, 总计56次, 累计扩张肺动脉260支, 每次扩张血管(5.14±2.36)支。共13例患者测定了6 min步行距离, 为(307±130)m。入选患者PTPA术后均自述体力明显改善, 尤其以第一次手术后为著。PTPA术后24周, 入选患者平均肺动脉压由基线的(40.11±7.55)mmHg(1 mmHg=0.133 kPa)...     

慢性阻塞性肺疾病相关性肺动脉高压的血流动力学和肺功能相关性研究

目的：观察 COPD 相关性肺动脉高压(pulmonary hypertension,PH)患者的血流动力学和肺功能的相关性。方法共入选右心导管确诊的 COPD 相关 PH 患者64例。根据平均肺动脉压和心指数分为2组：36例严重 COPD 相关 PH 和28例非严重 COPD 相关 PH。比较2组患者各项肺功能参数的差异,与血流动力学指标进行相关性分析。结果 COPD 相关 PH 患者的 FVC% pred[(55.03±22.2)%]、FEV1%pred 降低[(31.4±13.1)%];残气量%pred[(184.7±63.4)%]以及气道阻力%pred [(450.9±296.8)%]增高;并伴弥散量%pred[(58.5±31.1)%]降低。无论是肺容量通气指标、气道阻力指标还是弥散功能,严重 COPD 相关 PH 和非严重 PH 2组间差异均无统计学意义。严重 COPD 相关PH 低氧血症明显,PaO 2和 SaO 2较非严重 PH 组分别低10.6 mmHg 及6.3%(95% CI ,-16.1~-5.0;-9.1~-3.5;P <0.01)。COPD 相关 PH 的 PaO 2和 SaO 2和肺动脉平均压负相关。结论COPD 相关 PH 患者存在严重阻塞性通气功能障碍以及中度弥散功能障碍,血流动力学受损程度和两者无明显相关性。但严重 COPD 相关 PH 低氧血症更为显著,提示除了通气弥散功能外,肺血管因素有可能参与其中。