内皮素通路在靶向治疗慢性血栓栓塞性肺动脉高压中的作用及机制

正慢性血栓栓塞性肺动脉高压(chronic thrombembolic pulmonary hypertension,CTEPH)是肺动脉高压的一种疾病亚型,是指由来自静脉系统或右心的血栓,引起一次或反复发生的肺血栓栓塞导致血栓机化、肺动脉管腔狭窄甚至闭塞、肺血管重塑,从而引起肺动脉压力进行性升高,进一步发展可出现呼吸功能不全和右心衰竭并最终死亡的疾病。2015年欧洲心脏病学会/欧洲呼吸病学会联合颁布的《肺动脉高压诊断和     

次大面积肺栓塞患者溶栓或单纯抗凝治疗

肺血栓栓塞症(pulmonary thromboembolism,PTE)是一种相对常见的肺循环疾病,由于血栓栓子阻塞肺动脉血管床,可能造成致命性急性右心功能衰竭,但经过积极治疗,血栓栓塞开通后,急性右心功能衰竭可以逆转,因此急性肺血栓栓塞症是一种可以治疗和康复的疾病.     

肺栓塞合并中枢神经系统病变3例报道

<正>肺栓塞(PE)是指各种栓子阻塞肺动脉系统为发病原因的一组或临床综合征总称。其中99%的PE栓子是血栓,故也称为肺血栓栓塞症(PTE),多来自于下肢深静脉及盆腔静脉。静脉系统栓子理论上不能进入动脉系统,但PE发生后,可瞬时或长时间致右心系统压力升高,导致卵圆孔开放,脱落的栓子通过开放的卵圆孔由右心系统进入左心系统,进而导致动脉系统血栓栓塞,其中以神经系统特别是脑     

多层螺旋计算机断层肺动脉成像在慢性血栓栓塞性肺动脉高压及右心结构评价中的应用价值

目的探讨多层螺旋计算机断层肺动脉成像在慢性血栓栓塞性肺动脉高压及右心结构评价中的应用价值。方法对我院2016年4月至2017年4月收治的40例慢性血栓栓塞性肺动脉高压患者的临床资料进行统计分析,将这些患者纳入研究组,另选取我院同期接收的40例健康体检人员为对照组,对两组人员的右心结构CT检测结果、研究组患者右心结构CT和超声测量值结果及其相关性进行统计分析。结果研究组患者的右心结构CT检测结果中右房横径、右房上下径、右室基底段内径、RVAWT、dPA、rPA均显著长于对照组(P0.05),右心结构CT测量值结果中右房横径、右房上下径、右室基底段内径、RVAWT、dPA、右室横径/心脏横径均显著长于超声(P0.05),CT和超声测量值结果均呈显著的正相关关系(P0.05)。结论多层螺旋计算机断层肺动脉成像在慢性血栓栓塞性肺动脉高压及右心结构评价中具有较高的应用价值,值得在临床推广应用。     

慢性血栓栓塞性肺动脉高压的研究进展

1 概念及流行病学 慢性血栓栓塞性肺动脉高压(claronic throm boembolic pulmonary hypertension,CTEPH)是指肺动脉及其主要分支由于血栓栓塞所导致的血流受阻,长期不能缓解或进行性加重,导致肺动脉高压.CTEPH是肺血栓栓塞症中的一种特殊类型,是血栓不能完全溶解,或者是在深静脉血栓形成反复脱落的基础上继发反复多次栓塞肺动脉、血栓机化,肺动脉内膜慢性炎症并增厚,发展为慢性肺栓塞.CTEPH进一步发展可出现呼吸功能不全和右心衰竭.     

心房颤动是肺栓塞的危险因素吗?

心房颤动（房颤）患者容易在左心房形成血栓，进而导致体循环栓塞的发生。这一点早已为大规模的临床试验所证实并为广大临床医师所熟知。但是能否据此推论房颤亦有可能导致右心血栓形成并进而造成肺栓塞呢？要回答这一问题并不容易。原因是多方面的：由于解剖结构的关系，右心血栓难以通过常规检查发现，因此临床医师经常并不清楚房颤患者是否合并发生右心血栓；房颤患者接受抗凝治疗使得右心血栓的临床诊断率进一步下降；肺栓塞缺乏特征性临床表现，极易漏诊；肺栓塞患者也可能合并发生包括房颤在内的多种心律失常，致使两大疾病的因果关系变得更加错综复杂。因此，虽然临床意义不容低估，但是针对房颤是否是肺栓塞危险因素的研究一直相对较少。我们将为此作一简要综述。     

肺栓塞短期预后评估方法的研究进展

肺栓塞(pulmonary embolism,PE)是指肺外的栓子经静脉系统回流到右心,在肺动脉中堵塞,而引起的以肺循环障碍为基础的一系列临床病理生理综合征,包括肺血栓栓塞症、脂肪栓塞综合征、羊水栓塞、空气栓塞等。其中肺血栓栓塞症(pulmonary thromboembolism,PTE)是最常见的一种类型,为来自静脉系统或右心的血栓阻塞肺动脉或其分支所致疾病,以肺循环和呼吸功能障碍为其主要临床和病理生理特征,通常也简称肺栓塞[1]。     

慢性血栓栓塞性肺动脉高压

慢性血栓栓塞性肺动脉高压(CTEPH)是一种继发性肺动脉高压，它有自身的病因及发病机制，因而是可治疗的。针对其肺动脉高压及右心衰竭的表现，可以采取药物治疗以缓解症状；针对肺动脉内的残留机化血栓，可采取肺血栓动脉内膜切除术及球囊肺血管成形术；而且肺移植亦可用于该病的治疗。有资料显示，CTEPH发病率及死亡率均高。本文就流行病学、发病机制、诊断及治疗等进行综述。     

慢性血栓栓塞性疾病一例

肺栓塞是以各种栓子阻塞肺动脉或其分支引发的一组疾病或临床综合征的总称,最常见的是肺血栓栓塞症（PTE）。其中部分患者因血栓不溶、机化、肺血管重塑致血管狭窄或闭塞,导致肺血管阻力（PVR）增加,肺动脉压力进行性增高,最终可引起右心室肥厚和右心衰竭,称为慢性血栓栓塞性肺动脉高压（CTEPH）。国外经右心漂浮导管确诊的症状性...     

心房颤动与肺栓塞的关系及治疗

心房颤动(房颤)导致的血栓栓塞主要以动脉血栓栓塞为主,而肺栓塞作为重要的静脉血栓栓塞疾病之一,与房颤也存在一定关联。颤动的右心房同样可形成血栓并脱落致肺栓塞,并且房颤本身也会伴随机体高凝状态而导致静脉血栓形成风险升高。另外,肺栓塞引起的缺氧及右心压力的增加、扩张也是房颤发生的危险因素。对于合并房颤和肺栓塞的患者,抗栓仍是核心,但需注意个体化评估治疗。     

A case of vasospastic angina resulting from coronary emboli in a patient with prosthetic valves

In addition to coronary atherosclerotic disease, coronary thromboembolism can also lead to acute coronary syndromes. However, coronary thromboembolism due to prosthetic heart valves is very rare and not very well-known. It have been reported only a few cases. In this paper, we present a rare case with vasospastic angina pectoris secondary to coronary thromboembolism in a patient with prosthetic heart valves.     

Should Transesophageal Echocardiography Become a Routine Test in Patients with Suspected Pulmonary Thromboembolism?

Pulmonary thromboembolism presents in two clinical subsets: acute pulmonary embolism (PE) with or without right heart thrombi or paradoxical embolism and chronic thromboembolic pulmonary hypertension (CTEPH). Both PE and CTEPH have been underdiagnosed and carry high mortality rates. Acute massive PE is a hemodynamic entity leading to right ventricular overload readily identified with the use of transthoracic echocardiography. Transesophageal echocardiography (TEE) is a noninvasive bedside technique that has high diagnostic accuracy for the detection of central pulmonary thromboembolism. Due to the high prevalence of central pulmonary thromboembolism in acute PE, TEE is a useful method to provide the necessary proof for the institution of thrombolytic therapy. In the subset of patients with acute PE combined with right heart thrombi or paradoxical embolism, TEE is the technique of choice to guide surgery. CTEPH presents as primary pulmonary hypertension, but it has become a surgically curable disease. TEE is a fast, fairly sensitive, and highly specific diagnostic bedside modality to select surgical candidates with CTEPH. TEE should become a routine test in patients with suspected massive acute PE, suspected right heart thrombi, or paradoxical embolism associated with acute pulmonary embolism and in patients with primary pulmonary hypertension to select those having CTEPH who are suitable for surgery.     

Acute massive (pericardial effusive) pulmonary thromboembolism-pulmonary embolectomy revisited

The most recent patient in a 13-year experience with 14 patients suffering massive pulmonary thromboembolism requiring pulmonary thromboembolectomy is the focus of this report. This 40-year-old woman not only survived life-threatening acute hypoxemia and right heart failure, but was also found to have developed a unique transudative 700 cc pericardial effusion. Pulmonary artery pressure was 90/30 (mean 50 mmHg), accompanied by 17 mm right ventricular alternans. Systemic alternans and tamponade physiology were absent. This unusual natural model for acute right heart failure illustrates a novel mechanism for pericardial effusion physiology.     

肺心病合并肺血栓栓塞40例临床分析

目的 探讨COPD所致慢性肺原性心脏病并肺血栓栓塞（PTE）临床表现特点。方法 回顾性分析40例COPD所致肺心病合并PTE与单纯的COPD所致慢性肺心病40例进行对比。结果 肺心病合并肺栓塞组顽固性右心衰的发生率高、肺动脉压明显升高；氧分压明显下降，与单纯肺心病组比较，差异有显著性（P〈0．05）。结论 在诊断COPD所致慢性肺心病时，遇有难于解释的顽固性右心衰、高肺动脉压及低氧血症时应警惕合并PTE，尽快作胸部CT肺动脉造影或磁共振肺动脉造影（MRIPA）等检查以进一步明确诊断。     

Right heart thrombus: recognition, diagnosis and management

The clinical, echocardiographic, hemodynamic, angiographic and pathologic features of five patients who had right heart thrombus are presented and their management is discussed. Two modes of presentation were recognized. In four patients, right heart thrombus complicated peripheral venous thrombosis and was associated with major pulmonary thromboembolism and right heart obstruction. In the fifth, it complicated myocarditis with heart failure and appeared to cause right heart obstruction. Two-dimensional echocardiography was diagnostic of right heart thrombus in four patients and showed evidence of right heart dysfunction in those with major pulmonary thromboembolism. The diagnosis was confirmed at surgery in three patients and at autopsy in one. Three patients successfully underwent surgical removal of the thrombus followed by anticoagulation. One patient was treated successfully with anticoagulation alone. The only death occurred in the patient with myocarditis.     

Treatment of right heart thromboemboli

BACKGROUND: The presence of right heart thromboemboli complicating pulmonary thromboemboli carries with it an increased mortality rate compared to pulmonary thromboemboli alone, but little is known about the optimal management of this difficult clinical situation. This fact is highlighted in the case study of a patient with a 19-cm right atrial thrombus complicating bilateral pulmonary thromboemboli. STUDY OBJECTIVES: We sought to determine the effects of anticoagulation therapy, thrombolysis, and surgical embolectomy on mortality rate in patients with right heart thromboemboli. DESIGN: Retrospective analysis of all reported cases in the English language literature (1966 to 2000) of right heart thromboembolism in which age, sex, therapy, and outcome were reported. MEASUREMENTS AND RESULTS: We analyzed 177 cases of right heart thromboembolism. Pulmonary thromboembolism was present in 98% of the cases. The patients were evenly divided by gender with an average age of 59.8 years (SD, 16.6 years) years. Dyspnea (54.2%), chest pain (22.6%), and syncope (17.5%) were the most common presenting symptoms. The treatments administered were none (9%), anticoagulation therapy (35.0%), surgical procedure (35.6%), or thrombolytic therapy (19.8%). The overall mortality rate was 27.1%. The mortality rate associated with no therapy, anticoagulation therapy, surgical embolectomy, and thrombolysis was 100.0%, 28.6%, 23.8%, and 11.3%, respectively. Using multivariate modeling with survival as the primary outcome, age and gender were not associated with mortality rate, but thrombolytic therapy was associated with an improved survival rate (p < 0.05) when compared either to anticoagulation therapy or surgery. CONCLUSION: The presence of right heart thromboemboli may have diagnostic and therapeutic implications in pulmonary thromboembolism patients. A well-designed prospective, randomized trial is needed to determine the optimal treatment of right heart thromboemboli.     

The role of transesophageal echocardiography in the management of patients with acute and chronic pulmonary thromboembolism

Acute and chronic pulmonary thromboembolism carry high mortality. The role of transesophageal echocardiography (TEE), however, has not been well delineated in patients with suspected pulmonary thromboembolism. The aim of the present study was to demonstrate the value of Tee in patients with various clinical manifestations of pulmonary thromboembolic disease. Twelve patients--ten males and two females, age 47-85 years--are presented in whom central pulmonary thromboembolism was found by TEE. Six patients were referred for breathlessness and had moderate to severe pulmonary hypertension (PH) with (3) or without (1) right atrial thrombus or had right heart dilatation (1) or right ventricular myxoma (1) on transthoracic echocardiography (TTE). Thrombolysis (2), surgery (2), and heparin (2) treatment was performed without angiography. All but one patient recovered. Six patients had severe PH by TTE, one of them had a right atrial thrombus. Angiography was done in five patients in whom surgery was considered. Pulmonary thromboendarterectomy was successfully performed in two patients, it was contraindicated in two patients for advanced age or severe left ventricular dysfunction, both patients died during follow-up, and two patients were waiting for surgery. In conclusion: TEE has a definite role in the management of patients with acute pulmonary thromboembolism or in pulmonary embolism associated with right-sided intracardiac masses and in the selection of patients with PH for pulmonary thromboendarterectomy.     

白塞综合征合并心脏血栓八例临床分析

目的 通过分析白塞综合征合并心脏血栓的临床特点,提高对本病的认识.方法 总结分析1990年1月至201 1年1月北京协和医院确诊并住院治疗的8例白塞综合征合并心脏血栓患者的临床特点.结果 8例白塞综合征合并心脏血栓的患者占同期住院白塞综合征的1.2％( 8/667);其中男性5例,女性3例,发病年龄13 ～40岁,中位年龄28.5岁;心脏血栓多发生于右心内,常合并肺栓塞,抗心磷脂抗体均为阴性,ESR、C反应蛋白基本正常.8例患者中3例在诊治中曾被误诊(感染性心内膜炎、心脏肿瘤)或漏诊.2例患者接受了心脏取栓术,7例进行了抗凝及糖皮质激素+免疫抑制剂治疗.3例患者治疗后复查超声心动图见血栓缩小并机化.2例患者手术取栓后复查未再发现心脏内血栓.2例患者虽经泼尼松和免疫抑制剂治疗效果不佳,因经济困难自动出院.1例患者接受规律随诊,临床情况稳定,但未复查超声心动图,血栓情况不详.结论 对男性青年患者出现心脏血栓等表现,尤其心脏血栓出现在右心时,应考虑白塞综合征的可能;口腔溃疡、外阴溃疡、痤疮样皮疹、脓性丘疹等表现可帮助白塞综合征的诊断.     

Successful treatment of chronic pulmonary thromboembolism by long-term intermittent administration of milrinone,a phosphodiesterase-ill inhibitor

We report a 71-year-old man with right heart failure due to chronic pulmonary thromboembolism. Oral medications failed to control the condition but he was later successfully treated with intermittent administration of a PDE-III inhibitor for 2 years. Such therapy did not result in exacerbation of right heart failure but improvement of ANP/BNP levels to 32/104 pg/ml from a pretreatment value of 61/158 pg/ml, although no improvement was noted in pulmonary artery pressure or cardiac index. Intermittent administration of PDE-III inhibitors is a useful treatment for patients with right heart failure resistant to oral agents.     

Right heart thrombus-in-transit in a patient with Evans syndrome: A case report

Introduction:Right heart free-floating thrombus in the absence of structural heart disease or atrial fibrillation is rare. When it travels to the heart into the lung, called thrombus-in-transit, may cause cardiopulmonary collapse and sudden death. The clinical presentation varies from mild respiratory symptoms to sudden death; however, there are few clinical case reports of giant, free-floating thrombus in the right heart in an asymptomatic patient, and the optimal management options have not been established.Patient concerns:A 36-year-old Asian woman presented to the emergency department with complaints of worsening swelling of the left lower extremity over 12 hours.Diagnosis:Left leg deep vein thrombosis accompanied by an asymptomatic giant right atrial thrombus and pulmonary embolism with a rare autoimmune disease of Evans syndrome.Interventions:Emergent surgical thrombectomy under cardiopulmonary bypass for right atrial thrombus.Outcomes:The postoperative course was uneventful, and she was discharged on the eighth postoperative day with normal heart function and mild tricuspid regurgitation.Conclusion:An additional diagnostic workup in cases of deep vein thrombosis is necessary for the rapid diagnosis of right heart thrombus and pulmonary embolism without delay. This case report illustrates that early recognition of venous thromboembolism and emergent thrombectomy of right heart thrombus-in-transit is crucial to prevent mortality.