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新治疗方法的进展

慢性血栓栓塞性肺动脉高压是由于肺动脉血栓未完全溶解导致的一类毛细血管前性肺动脉高压.肺动脉内膜剥脱术是目前慢性血栓栓塞性肺动脉高压的首选治疗方案.但很多患者因远端血管病变或严重合并症而无法手术,或手术后有持续性肺动脉高压,球囊肺血管成形术和靶向药物的发展为这些患者的治疗带来了新的希望.现就慢性血栓栓塞性肺动脉高压新疗法...     

慢性血栓栓塞性肺动脉高压的介入治疗

慢性血栓栓塞性肺动脉高压(CTEPH)是肺栓塞的潜在致命结果。目前肺动脉内膜剥脱术(PEA是首选的治疗方法,但多种因素限制了其临床应用和开展。对于不能行PEA治疗的CTEPH患者,经皮球囊肺动脉成形术是近年来发展迅速的一种能够改善患者临床症状、降低肺动脉压力和肺血管阻力并改善患者预后的新的介入治疗方法。     

肺动脉高压的治疗

导致肺动脉高压的原因很多:如二尖瓣狭窄或肺静脉闭塞使肺静脉回流受阻以致被动地形成肺动脉高压;当房间隔缺损;巨大肺动脉瘘以及其它先天性心血管畸形,肺血流量增加,出现高动力状态的肺动脉高压;也可因肺动脉血栓形成、巨大肺动脉栓塞或广泛多发肺小动脉栓塞,或先天性肺动脉缺如以及肺动脉狭窄造成肺动脉侧的阻塞导致肺动脉压力升高;肺血管本身的疾病如肺血管炎或原发性肺动脉高压等由于较小的血管血流受阻引起肺动脉高压;慢性高原病或睡眠呼吸障碍等因缺氧导致肺血管收缩使肺血管阻力增加肺动脉压力增高,而慢性气管炎肺气肿(即慢性阻塞性肺疾病COPO),或慢     

《中国肺动脉高压诊断与治疗指南(2021版)》解读：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)是一种急性肺血栓栓塞症的远期并发症。2021年1月,《中国肺动脉高压诊断与治疗指南(2021版)》发布,将CTEPH作为单独章节,从CTEPH早期识别、诊断及治疗三个方面出发,明确了如何利用影像学检查早期识别CTEPH,提出了肺动脉血栓内膜剥脱术、靶向药物治疗、球囊肺动脉成形术的适...     

书讯

<正>由首都医科大学附属北京安贞医院心脏外科中心,甘辉立教授主编的《肺动脉栓塞学》一书,已由人民军医出版社出版,其ISBN 978-7-5091-8006-8,有感兴趣者可在各大书店订购。全书原价为98元人民币。该书为中文著作,全书310页,附图60余帧,为有关急性肺动脉栓塞和慢性血栓栓塞性肺动脉高压内科治疗及外科治疗的专著。急性肺动脉栓塞和慢性血栓栓塞性肺动脉高压是严重威胁健康的疾病,死亡率和病残率都很高。抗凝治疗和溶栓治疗、肺动脉取栓术和肺动脉血栓内膜剥脱术治疗,分别是治疗急性肺动脉栓塞和慢性血栓栓塞性肺动脉高压的有效治疗方法,可     

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…     

慢性血栓栓塞性肺动脉高压的介入治疗及策略

<正>慢性血栓栓塞性肺高血压(chronic thromboembolic pulmonaryhypertension,CTEPH)是未溶解的血栓发生机化、内膜纤维性增生，近端肺血管腔狭窄甚至闭塞、远端肺血管重构，引起肺动脉压力及肺血管阻力进行性升高，右心负荷逐渐增加，最终导致右心衰竭甚至死亡的一组病理生理学复杂的临床综合征^[1]。其主要临床表现为呼吸困难、乏力、活动耐力减低。依照我国2021年肺高血压指南的临床分类，CTEPH属于第四类肺动脉阻塞性疾病导致的肺高血压；按照血流动力学分类，CTEPH属于毛细血管前性肺动脉高压。     

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。     

慢性栓塞性肺动脉高压的外科治疗

一、背景慢性栓塞性肺动脉高压 (ｃｈｒｏｎｉｃｔｈｒｏｍｂｏｅｍｂｏｌｉｃｐｕｌｍｏｎａｒｙｈｙｐｅｒｔｅｎｓｉｏｎ ,ＣＴＥ ＰＨ )是由于肺动脉内反复栓塞和血栓形成而造成的肺动脉高压。既可由急性肺动脉栓塞演变而来 ,也可因下肢静脉血栓等反复栓塞肺动脉所致。慢性栓塞性肺动脉高压呈渐进性 ,最终造成右心衰竭和呼吸衰竭死亡。其自然预后与肺平均动脉压 (ｍＰＡＰ)有关。ｍＰＡＰ >30ｍｍＨｇ(1ｍｍＨｇ =0 .133ｋＰａ) ,5年生存率为 30 % ;ｍＰＡＰ >50ｍｍＨｇ ,5年生存率为 10 % [1] 。内科对慢性栓塞性肺动脉高压的治疗多是…     

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.     

Enfermedad tromboembólica crónica pulmonar

Persistent thrombotic lesions are common in patients with pulmonary embolism. These lesions occur on a clinical spectrum, ranging from an asymptomatic course with complete functional recovery to chronic thromboembolic pulmonary hypertension. The concept of chronic thromboembolic disease has emerged in recent years to describe a subgroup of patients with persistent thrombotic lesions who have symptoms on exertion and pulmonary vascular dysfunction, but no pulmonary hypertension at rest. The prevalence of this entity is unknown and the criteria for diagnosing it are not defined. The aim of this article is to analyze post- pulmonary embolism sequelae and review existing evidence on chronic thromboembolic disease, with special emphasis on its diagnosis and therapeutic approach.     

Post splenectomy related pulmonary hypertension

Splenectomy predisposes patients to a slew of infectious and non-infectious complications including pulmonary vascular disease. Patients are at increased risk for venous thromboembolic events due to various mechanisms that may lead to chronic thromboembolic pulmonary hypertension (CTEPH). The development of CTEPH and pulmonary vasculopathy after splenectomy involves complex pathophysiologic mechanisms, some of which remain unclear. This review attempts to congregate the current evidence behind our understanding about the etio-pathogenesis of pulmonary vascular disease related to splenectomy and highlight the controversies that surround its management.     

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.     

Is pulmonary embolism a common cause of chronic pulmonary hypertension? Limitations of the embolic hypothesis.

The hypothesis that chronic thromboembolic pulmonary hypertension results from unresolved pulmonary embolism has strongly influenced the diagnosis and management of this disease since the 1960s. However, it is nearly impossible to induce chronic pulmonary hypertension in any animal species by means of repeated embolization of thrombotic material. The haemodynamic effects of thrombotic pulmonary embolism of different degrees of magnitude have also been studied in humans and there is little to suggest that chronic pulmonary hypertension is a likely long term outcome. Furthermore many conditions which predispose to venous thromboembolism do not appear to cause thromboembolic pulmonary hypertension. Other arteriopathic and atherosclerotic risk factors, are found in patients with chronic thromboembolic pulmonary hypertension, but not in those with venous thrombosis, suggesting that these may be unrelated conditions. Thrombosis in situ of the pulmonary arteries is common in severe pulmonary hypertension of any cause. Such thrombosis cannot usually be distinguished from pulmonary embolism. It is hypothesized that in situ thrombosis and pulmonary arteriopathy are common causes of vascular occlusion which is usually diagnosed as "chronic thromboembolic pulmonary hypertension" and that venous thromboembolism is unlikely to be a common cause of chronic pulmonary hypertension. It is further hypothesized that pulmonary embolism is seldom the sole cause of "chronic thromboembolic pulmonary hypertension".     

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.     

吸入伊洛前列素对不同类型肺动脉高压患者氧动力学的影响

目的 分析雾化吸入伊洛前列素对动脉型肺动脉高压(PAH)及慢性血栓栓寒性肺动脉高压(CTEPH)患者的氧动力学的急性影响.方法 顺序收集北京朝阳医院2006年6月至2009年1月连续收治的明确诊断为PAH的患者22例及CTEPH患者24例,均行右心导管检查,监测基线状态及吸人伊洛前列素20μg后的即刻血流动力学特征,同步抽取肺动脉及股动脉血行血气分析,计算氧动力学参数.结果 基线状态PAH及CTEPH患者的PaO2分别为(63±10)mm Hg(1 mm Hg=0.133 kPa)及(59 ±10)mm Hg,氧输送指数(DO2I)分别为(458±136)ml·min-1·m-2及(386 ±92)ml·min-1·m-2,氧消耗指数分别为(135±53)ml·min-1·m-2及(131±43)ml·min-1·m-2.吸入伊洛前列素后即刻,2组患者肺内分流率均显著升高(均P<0.01),P4O2显著升高(均P<0.01);动脉血氧含量均显著降低(均P<0.05);混合静脉血氧合指标及氧摄取率无明显变化;DO2I无明显增加,氧消耗指数出现不同程度降低;2组患者各项氧动力学参数变化幅度无差异.基线状态CTEPH患者混合静脉血氧饱和度(SvO2)、静脉血氧含量(CvO2)及DO2I均显著低于PAH患者(均P<0.05).吸药后,CTEPH患者PaO2、SvO2及CvO2均显著低于PAH患者(均P<0.05).结论 PAH及CTEPH患者存在低氧血症及氧动力学异常;雾化吸入伊洛前列素后患者肺内分流增加,氧动力学状态无改善.CTEPH患者的氧动力学状态较PAH患者更差,应用伊洛前列素时需加强氧合功能监测,必要时给予氧疗支持.     

Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension.

For chronic thromboembolic pulmonary hypertension not amenable to pulmonary endarterectomy, effective medical therapy is desired. In an open-label uncontrolled clinical trial, 104 patients (mean +/- sem age 62 +/- 11 yrs) with inoperable chronic thromboembolic pulmonary hypertension were treated with 50 mg sildenafil t.i.d. At baseline, patients had severe pulmonary hypertension (pulmonary vascular resistance 863 +/- 38 dyn.s.cm(-5)) and a 6-min walking distance of 310 +/- 11 m. Eight patients were in World Health Organization functional class II, 76 in class III and 20 in class IV. After 3 months' treatment, there was significant haemodynamic improvement, with reduction of pulmonary vascular resistance to 759 +/- 62 dyn.s.cm(-5). The 6-min walking distance increased significantly to 361 +/- 15 m after 3 months' treatment, and to 366 +/- 18 m after 12 months' treatment. A subset of 67 patients received a single dose of 50 mg sildenafil during initial right heart catheterisation. The acute haemodynamic effect of this was not predictive of long-term outcome. In this large series of patients with inoperable chronic thromboembolic pulmonary hypertension, open-label treatment with sildenafil led to significant long-term functional improvement. The acute effect of sildenafil may not predict the long-term outcome of therapy.     

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.