慢性肺动脉栓塞的外科治疗

慢性肺动脉栓塞性肺动脉高压是血管腔内血栓机化、纤维化增生、血管腔狭窄、肺血管重构所致的一种预后较差的疾病,常常引发进展性的肺血管阻力增大和右心功能的衰竭。肺动脉内膜剥脱术已经成为治疗慢性肺动脉栓塞性肺动脉高压的有效方法之一,现就慢性肺动脉栓塞性肺动脉高压病人选择外科治疗和术后处理做一综述。     

慢性血栓栓塞性肺动脉高压的外科治疗

慢性血栓栓塞性肺动脉高压是一种自然预后极差的疾病,疾病早期容易误诊或漏诊.目前肺动脉血栓内膜剥脱术是慢性血栓栓塞性肺动脉高压患者的首选治疗方案,该手术效果明显,术后早期可明显降低肺动脉压力和肺血管阻力,改善血流动力学和患者生活质量.术前评估患者是否已存在严重的继发性肺小血管病变至关重要.术中建立正确的内膜剥离层面和完整的剥离血栓及机化内膜是外科技术的关键.     

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.     

肺动脉血栓内膜剥脱术后再灌注肺水肿的进展

肺血栓栓塞症是一种常见、多发且病死率和致残率高的疾病。大多数急性肺动脉血栓栓塞经及时的溶栓抗凝等治疗和 (或 )自身的纤溶系统能将血栓不同程度地溶解 ,另有0 1%～ 0 2 %的患者因血栓在急性期未能溶解或栓塞反复发生进而发展成慢性栓塞性肺动脉高压。慢性栓塞性肺动脉高压溶栓无效 ,抗凝、扩血管治疗效果不佳 ,其病理过程多呈进行性加重或稳定一段时间后再次加重 ,自然预后差。肺动脉平均压 >3 0ｍｍＨｇ(1ｍｍＨｇ =0 13 3ｋＰａ)的慢性栓塞性肺动脉高压患者 5年生存率为 3 0 % ,肺动脉平均压 >5 0ｍｍＨｇ者仅为 10 %。肺动脉血…     

老年肺血栓栓塞症患者血流动力学的变化及影响因素

目的探讨老年肺血栓栓塞症患者血流动力学的变化及影响因素。方法选择70岁以上老年肺血栓栓塞症患者100例作为肺血栓栓塞症组,70岁以上老年健康体检者100例作为对照组。肺血栓栓塞症组患者根据是否合并肺动脉高压分为肺动脉高压组(59例)和非肺动脉高压组(41例)。收集患者的临床资料及平均动脉压(MAP)、心脏指数(CI)、每搏输出量(CO)、每搏指数、心率、胸腔静脉血压、体血管阻力、肺血管阻力等血流动力学指标。结果肺血栓栓塞症组MAP、CI、CO、每搏指数明显均明显低于对照组(P<0.05),肺血栓栓塞症组心率、胸腔静脉血压、体血管阻力、肺血管阻力均明显高于对照组(P<0.05)。肺动脉高压组年龄明显高于非肺动脉高压组,血小板计数低于非肺动脉高压组,女性、晕厥、V1~3 T波倒置比例明显高于非肺动脉高压组;肺动脉高压组吸烟、高血压、脑卒中、癌症、外伤或手术、呼吸困难、胸膜炎性胸痛、咯血、胸闷、心悸、咳嗽、D-二聚体、右束支传导阻滞、深静脉血栓形成的比例与非肺动脉高压组比较均无统计学差异(P>0.05)。Logistic多因素回归分析结果显示:性别和年龄是肺血栓栓塞症合并肺动脉高压的独立危险因素(P<0.05)。结论老年肺血栓栓塞症患者存在血流动力学改变,性别和年龄是老年肺血栓栓塞症合并肺动脉高压的独立危险因素。     

慢性血栓栓塞性肺动脉高压

慢性血栓栓塞性肺动脉高压是肺动脉高压的一大类,是一种自然预后极差的疾病,目前认为机化血栓与肺血管重塑是其发病的重要组成部分。近年来随着基础和临床研究的发展,对慢性血栓栓塞性肺动脉高压的认知有所提高。随着外科手术治疗、介入治疗和新型靶向药物的临床应用,其预后有了明显的改善。现对慢性血栓栓塞性肺动脉高压的发病机制、病理、诊断和治疗等方面进行综述。     

肺血栓栓塞症的诊疗现状及展望

肺血栓栓塞症具有高发病率、高误诊率和高病死率。其栓子主要来源于下肢深静脉血栓，其和下肢深静脉血栓是一个疾病的两个阶段。该病各种临床表现多与缺氧及肺动脉高压导致的血流动力学不稳定密切相关。常用的无创诊断手段包括心电图、胸部X线、D二聚体定量、血气分析、心脏超声、CTPA、MRA及核素肺通气灌注扫描等。有创检查主要是肺血管造影。CTPA在肺动脉主干、叶、段水平的肺栓塞诊断敏感性为96％，特异性为92％，已经逐步被认为是取代肺血管造影的无创检查之首选。肺血栓栓塞症的主要治疗包括病因干预、抗栓治疗及并发症对症支持等治疗。对急性大面积血栓患者，溶栓治疗疗效优于单纯抗凝。抗凝治疗是肺血栓栓塞症的基础治疗，常用药物有普通肝素、低分子肝素和华法林。普通肝素治疗强调早达标和稳定达标，在治疗急性大面积血栓中疗效优于低分子肝素，后者在治疗急性非大面积血栓中疗效等于甚至优于普通肝素。华法林半衰期长，起效慢，用于预防慢性血栓形成，使用疗程至少3个月。影响肺血栓栓塞症的预后因素为急性右心衰和慢性肺动脉高压。急性肺血栓栓塞症患者未能及时诊治、病程慢性迁延、溶栓治疗无效、多发的反复的亚型肺小血栓导致压力升高等因素均容易导致慢性血栓栓塞性肺动脉高压的发展。降低肺动脉高压的药物可能有助于改善预后。     

慢性血栓栓塞性肺高血压的诊治进展

慢性血栓栓塞性肺高血压(CTEPH)是各种原因导致肺动脉内血栓形成或栓塞并不完全溶解、机化致使肺动脉慢性阻塞,肺血管发生重构,导致肺血管阻力持续升高并肺动脉压力增高的一类疾病.CTEPH是一种进展性疾病,病理生理机制复杂,患者晚期出现右心衰竭,死亡率高.由于患者临床表现(气短、胸闷、水肿、咯血)缺乏特异性,诊断难度较大...     

经皮肺动脉成形术在慢性血栓栓塞性肺动脉高压应用中的研究进展

慢性血栓栓塞性肺动脉高压是由肺动脉血栓机化并堵塞肺动脉引起的一类严重心肺血管疾病,预后极差。目前的治疗方式主要有药物、肺动脉内膜剥脱术及经皮肺动脉成形术。对于药物治疗效果不佳、无法耐受或不愿意行肺动脉内膜剥脱术的患者,经皮肺动脉成形术提供了一种新的选择。     

如何在实践中理解及运用肺栓塞指南中危肺动脉血栓栓塞症患者的溶栓治疗

中危组肺动脉血栓栓塞症的患者溶栓治疗已经争论很多年,尤其是溶栓治疗为患者带来益处/风险比得到重视,慢性血栓栓塞性肺动脉高压是决定肺动脉血栓栓塞患者预后的重要因素,所以残余血栓的研究对慢性血栓栓塞性肺动脉高压的影响更值得关注.     

Chronic Thromboembolic Pulmonary Hypertension Medical Management

Chronic thromboembolic pulmonary hypertension (CTEPH) is a common long-term complication of pulmonary embolism characterized by thromboembolic obstruction of the pulmonary arteries, vascular arteriopathy, vascular remodeling, and ultimately pulmonary hypertension (PH). Although pulmonary endarterectomy (PEA) surgery is the standard of care, approximately 40% of patients in the international CTEPH registry were deemed inoperable. In addition to lifelong anticoagulation, the cornerstone of PH-specific medical management is riociguat, a soluble guanylate cyclase stimulator. Medical management should be started early in CTEPH patients and may be used as a bridge to PEA surgery or balloon pulmonary angiography. Medical management is indicated for inoperable CTEPH patients and patients who have recurrence of PH after PEA surgery.     

Balloon Pulmonary Angioplasty (Percutaneous Transluminal Pulmonary Angioplasty) for Chronic Thromboembolic Pulmonary Hypertension: A Japanese Perspective

In recent years, therapeutic options for patients with chronic thromboembolic pulmonary hypertension (CTEPH) have expanded with the development of catheter-based interventional therapy, namely balloon pulmonary angioplasty (BPA), also called percutaneous transluminal pulmonary angioplasty. For CTEPH patients with technically inoperable disease or with an unfavorable risk-to-benefit ratio for surgical pulmonary endarterectomy, BPA is an important alternative therapeutic strategy. One important treatment goal of BPA should be the relief of pulmonary hypertension. However, the indications for BPA in specialized Japanese centers currently go beyond the sole indication of relieving pulmonary hypertension. BPA is currently limited to specific institutes and experienced operators, which allows better management of its associated complications of reperfusion pulmonary edema and vascular injury using various strategies based on past experiences. This article discusses the latest indications and treatment goals of BPA and the current flow diagram for therapeutic decision-making in CTEPH, and summarizes the factors to be considered when performing BPA, from a Japanese perspective.     

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.     

Prospective cardiopulmonary screening program to detect chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism

Background

Chronic thromboembolic pulmonary hypertension after pulmonary embolism is associated with high morbidity and mortality. Understanding the incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism is important for evaluating the need for screening but is also a subject of debate because of different inclusion criteria among previous studies. We determined the incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism and the utility of a screening program for this disease.

Design and Methods

We conducted a cohort screening study in an unselected series of consecutive patients (n=866) diagnosed with acute pulmonary embolism between January 2001 and July 2007. All patients who had not been previously diagnosed with pulmonary hypertension (PH) and had survived until study inclusion were invited for echocardiography. Patients with echocardiographic suspicion of PH underwent complete work-up for chronic thromboembolic pulmonary hypertension, including ventilation-perfusion scintigraphy and right heart catheterization.

Results

After an average follow-up of 34 months of all 866 patients, PH was diagnosed in 19 patients by routine clinical care and in 10 by our screening program; 4 patients had chronic thromboembolic pulmonary hypertension, all diagnosed by routine clinical care. The cumulative incidence of chronic thromboembolic pulmonary hypertension after all cause pulmonary embolism was 0.57% (95% confidence interval [CI] 0.02–1.2%) and after unprovoked pulmonary embolism 1.5% (95% CI 0.08–3.1%).

Conclusions

Because of the low incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism and the very low yield of the echocardiography based screening program, wide scale implementation of prolonged follow-up including echocardiography of all patients with pulmonary embolism to detect chronic thromboembolic pulmonary hypertension does not seem to be warranted.     

Advances in understanding the pathogenesis of chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) comprises organizing thrombotic obstructions in the pulmonary arteries. While roughly 40% of CTEPH cases are not preceded by a venous thromboembolic event, 0·1–5·1% of acute pulmonary thromboemboli evolve into organized obstructions of the pulmonary artery. In patients with predominantly proximal disease, surgical pulmonary endarterectomy provides a potential cure of the disease. For years, the scientific debate of CTEPH was mainly focused around its thromboembolic nature because of striking dissimilarities to classical venous thromboembolism, for example, the lack of risk factors for venous thrombosis, the lack of clinically apparent pulmonary embolism in many patients, the difficulty to reproduce the disease in animal models of thrombosis, and the nature of the pulmonary vascular obstructions that are dissociated from the degree of hemodynamic compromise. Recent studies have confirmed an association between venous thromboembolism and the evolution of CTEPH, and have shed light on disease‐modifying conditions.     

Dyspnea in antiphospholipid syndrome: Beyond pulmonary embolism

Pulmonary embolism due to primary antiphospholipid syndrome is rarely associated with chronic thromboembolic pulmonary hypertension, and therefore according to the latest guidelines on pulmonary hypertension, routine screening is not recommended. We describe a young patient with a late diagnosis of chronic thromboembolic pulmonary hypertension in the context of pulmonary embolism, primary antiphospholipid syndrome and suboptimal anticoagulation. Of note, mild cardiopulmonary symptoms were consistently misattributed to a depressive disorder because physical examination was normal, serial Doppler echocardiography failed to show pulmonary hypertension, and all other diagnostic tests were normal. Once symptoms became severe, positive screening tests led to the correct diagnosis and surgical referral, and bilateral pulmonary endarterectomy was successfully performed. This case demonstrates the need for extra awareness in patients with antiphospholipid syndrome and pulmonary embolism.     

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.     

Pulmonary thromboendarterectomy surgery

Considerable progress has been made over the past decade in understanding the etiology, prevalence, natural history, and therapeutic approach to chronic thromboembolic pulmonary hypertension. Pulmonary endarterectomy is now widely recognized as the definitive treatment for chronic pulmonary hypertension resulting from thromboembolic disease. This article focuses on the surgical treatment of chronic thromboembolic pulmonary hypertension.     

Surgical treatment of chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension is a commonly overlooked cause of physical incapacity and dyspnoea, with a higher incidence than is generally appreciated and a poor prognosis. The diagnosis can be suspected based on echocardiographic examinations and ventilation perfusion scanning of the lung. Pulmonary angiography still remains the gold standard for the diagnosis of thromboembolic pulmonary hypertension and the assessment of operability. Pulmonary endarterectomy is a complex surgical procedure, which provides permanent relief of thromboembolic pulmonary hypertension and cure for most of the patients. The operation resembles a true endarterectomy of the pulmonary artery branches using extracorporeal circulation and periods of hypothermic circulatory arrest. In experienced centres, the operative risk has been decreased to an acceptable level. Following successful surgery, long-term survival and quality of life are excellent. Earlier referral to surgery might avoid the occurrence of a secondary vasculopathy in the unaffected areas of the pulmonary vascular tree, and therefore further improve early and late results. A multidisciplinary approach and surgical experience are the prerequisites for success of this demanding procedure.