慢性血栓栓塞性肺动脉高压:构建动物模型的研究进展

慢性血栓栓塞性肺动脉高压(CTEPH)被认为是急性肺血栓栓塞症的一种长期并发症,然而,研究表明,部分确诊为CTEPH的患者并无症状性肺血栓栓塞的既往病史,提示肺血栓栓塞可能并不是CTEPH的唯一病因.尽管近年来对肺动脉高压的研究取得了广泛的进展,但由于缺乏对CTEPH病理生理学的相关研究,此类患者的综合治疗仍面临许多挑...     

《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...     

慢性血栓栓塞性肺动脉高压的误诊分析

慢性血栓栓塞性肺动脉高压(CTEPH)是指肺动脉及其主要分支由于血栓栓塞导致的血流受阻,长期不能缓解或进行性加重,导致肺动脉高压。CTEPH的误诊率很高,笔者对近3年所收治的7例CHEPH患者进行误诊分析,以提高对CTEPH的认识。1对象与方法近3年我院心内科确诊的CTEPH患者共7例,男2例,女5例。年龄40~66(52±9.52)岁。均经螺旋CT肺血管造影(CTPA)和肺通气灌注扫描证实的至少有1个肺段以上的肺栓塞。患者均经过超声多普勒检测肺动脉收缩压>30mmHg(1mmHg=0·133kPa)以上,均排除其他非血栓栓塞性肺动脉高压。2结果症状体征:主要症状有…     

球囊肺动脉成形术治疗肺动脉血栓内膜剥脱术后残余/复发肺动脉高压的疗效和安全性研究

目的:对球囊肺动脉成形术(BPA)治疗慢性血栓栓塞性肺动脉高压(CTEPH)患者肺动脉血栓内膜剥脱术(PEA)术后残余/复发肺动脉高压的有效性和安全性进行评估。方法:本研究为单中心病例对照研究。采用非随机抽样的方法纳入2004年11月至2021年12月于首都医科大学附属北京朝阳医院行PEA的CTEPH患者76例,其中发...     

经皮腔肺动脉成形术在慢性血栓栓塞性肺动脉高压中的应用

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)是肺高血压(pulmonary hypertension,PH)非常重要和常见的类型[1],是以反复性未溶解肺动脉栓子和(或)进行性肺动脉血栓形成及瘢痕形成,以及肺动脉重塑所致肺动脉阻塞为特征的进行性致命性疾病。右心导管(right heart chatheterization,RHC)是诊断CTEPH的"金标准"[2],肺动脉(主干、叶段、节段、亚     

第六届肺动脉高压大会专家意见解读列之四——慢性血栓栓塞性肺动脉高压诊治策略更新

慢性血栓栓塞性肺动脉高压（CTEPH）是肺栓塞的并发症,其诊断和治疗需要多学科专家参与。2018年,第6届 世界肺动脉高压大会（WSPH）对CTEPH的认识和治疗进行了更新,强调早期识别和准确诊断的关键诊断步骤,提出 了新的治疗流程。文章旨在结合临床实践对此部分专家共识进行解读。     

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。     

慢性血栓栓塞性肺动脉高压机制研究进展

慢性血栓栓塞性肺动脉高压（chronic thromboembolic pulmonary hypertension,CTEPH）是急性肺栓塞（pulmonary embolism,PE）或肺动脉原位血栓形成的长期后果,由于种种原因血栓未溶解而持续存在,通过机化、纤维化造成肺动脉狭窄性重塑,从而引起肺动脉高压。CTEPH的发病机制尚未完全阐明,目前认为,肺动脉内皮功能障碍、肺动脉血管重塑在CTEPH的进展中发挥了重要的作用。     

慢性血栓栓塞性肺动脉高压的诊疗进展

慢性血栓栓塞性肺动脉高压(chronic thromboembolic pulmonary hypertension,CTEPH),属于肺动脉高压分型的第四大类,是目前唯一可能治愈的肺动脉高压,不干预则远期预后不佳,故明确诊断和积极治疗非常重要。对于CTEPH的诊断,肺通气灌注显像较CT肺动脉造影有更高的敏感性。CTEPH的预后与其治疗决策密切相关:所有患者都需要终身抗凝,利奥西呱是目前唯一获批用于CTEPH治疗的靶向药物。能否手术治疗取决于CTEPH的血栓机化部位:对于血栓栓塞在肺动脉近端,可行肺动脉内皮剥脱,其治愈率高,术后恢复以及长期预后往往较为理想;如果病变部位在中段,可以尝试进行肺动脉球囊扩张,球囊扩张往往需要分次逐步进行,扩张后患者活动耐量有显著提升,此外球囊扩张也用于如外科内皮剥脱后残余肺动脉高压或肺动脉高压复发;如果病变部位在肺血管末端,则只能选择药物保守治疗。多种治疗手段联合治疗或为未来CTEPH治疗的发展方向。     

慢性血栓栓塞性肺动脉高压机制研究进展

慢性血栓栓塞性肺动脉高压(chronic thromboembolic pulmonary hypertension,CTEPH)是急性肺栓塞(pulmonary embolism,PE)或肺动脉原位血栓形成的长期后果,由于种种原因血栓未溶解而持续存在,通过机化、纤维化造成肺动脉狭窄性重塑,从而引起肺动脉高压.CTEPH的发病机制尚未完全阐明,目前认为,肺动脉内皮功能障碍、肺动脉血管重塑在CTEPH的进展中发挥了重要的作用.     

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.     

Occlusion pressure analysis role in partitioning of pulmonary vascular resistance in CTEPH

Flow-directed pulmonary artery occlusion is posited to enable partitioning of vascular resistance into small and large vessels. As such it may have a role in assessment for pulmonary endarterectomy. To test if the occlusion technique distinguished small from large vessel disease we studied 59 subjects with chronic thromboembolic pulmonary hypertension (CTEPH), idiopathic pulmonary arterial hypertension (IPAH), and connective tissue disease (CTD)-associated PAH. At right heart catheterisation, occlusion pressures were recorded. With fitting of the pressure decay curve, pulmonary vascular resistance was partitioned into downstream (small vessels) and upstream (large vessels, Rup). 47 patients completed the study; 14 operable CTEPH, 15 inoperable CTEPH, 13 idiopathic or CTD-PAH and five post-operative CTEPH. There was a significant difference in mean Rup in the proximal operable CTEPH group 87.3 (95% CI 84.1-90.5); inoperable CTEPH mean 75.8 (95% CI 66.76-84.73), p=0.048; and IPAH/CTD, mean 77.1 (95% CI 71.86-82.33), p=0.003. Receiver operating characteristic curves to distinguish operable from inoperable CTEPH demonstrated an area under the curve of 0.75, p=0.0001. A cut-off of 79.3 gave 100% sensitivity (95% CI 73.5-100%) but 57.1% specificity (95% CI 28.9-82.3%). In a subgroup analysis of multiple lobar sampling there was demonstrable heterogeneity. Rup is significantly increased in operable proximal CTEPH compared with non-operable distal CTEPH and IPAH/CTD-PAH. Rup variability in patients with CTEPH and PAH is suggestive of pathophysiological heterogeneity.     

Therapie der chronischen Thromboembolie (CTEPH)

Chronic thromboembolic pulmonary hypertension (CTEPH) is a frequent cause of pulmonary hypertension, however, it still is underdiagnosed. Up to 4% of all individuals that survive pulmonary embolism develop CTEPH. Increasing insights into the pathophysiology of CTEPH indicate a distinct overlap with pulmonary arterial hypertension (PAH). Pulmonary endarterectomy (PEA), which lowers pulmonary resistance and can lead to normalization of hemodynamics, is the treatment of choice for eligible patients, however, only a proportion of patients fulfill the criteria for surgical intervention. Patients who are not eligible for PEA may benefit from specific medical therapy for pulmonary hypertension, as do patients before and after PEA with significant peripheral involvement of the pulmonary vasculature. Direct evidence from clinical trials in CTEPH is promising, but is to date limited. Further studies are necessary to define criteria for specific medical therapy for CTEPH.     

An Update on the Management of Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but life-threatening form of pulmonary artery hypertension that is defined as a mean arterial pulmonary pressure greater than 25 mm Hg that persists for more than 6 months following anticoagulation therapy in the setting of pulmonary emboli. CTEPH is categorized by the World Health Organization as group IV pulmonary hypertension and is thought to be due to unresolved thromboemboli in the pulmonary artery circulation. Among the 5 classes of pulmonary hypertension, CTEPH is unique in that it is potentially curable with the use of pulmonary thromboendarterectomy surgery. Despite an increasing array of medical and surgical treatment options for patients with CTEPH over the past 2 decades, patients commonly present with advanced disease and carry a poor prognosis, thus, the need for early diagnosis and appropriate referral to an expert center. This review article first highlights the epidemiology, pathophysiology, and clinical presentation of CTEPH. The article then provides diagnostic and therapeutic algorithms for the management of the patient with suspected CTEPH.     

慢性血栓栓塞性肺动脉高压诊疗的研究进展

慢性血栓栓塞性肺动脉高压(CTEPH)是急性肺栓塞最严重的并发症,由于缺乏特异性的临床表现,常常会漏诊,造成患者死亡。近年来,虽然多种影像技术已应用于CTEPH 患者,但在CTEPH 的诊断、预后及治疗的选择决策上仍有不确定性,熟练掌握各类影像技术特点,可提升利用率。目前肺动脉内膜剥脱术是治疗CTEPH 的首选方式,对于无法手术治疗的患者,经皮肺动脉成形术可作为一种新的治疗选择。本文主要综述了影像学检查在CTEPH 的诊断、治疗及预后评估中的研究进展。     

Chronic Thromboembolic Pulmonary Hypertension: A Comprehensive Review and Multidisciplinary Approach to Surgical Treatment

Chronic thromboembolic pulmonary hypertension (CTEPH) is an underdiagnosed and undertreated sequelae of acute pulmonary embolism. In this comprehensive review, we provide an introductory overview of CTEPH, highlight recent advances in its diagnostic imaging, and describe the surgical technique for pulmonary thromboendarterectomy (PTE), the only established curative treatment for CTEPH. We also discuss the emerging role of balloon pulmonary angioplasty, both independently and combined with PTE, for patients with inoperable, residual, or refractory pulmonary hypertension post PTE. Finally, we stress the importance of a specialized multidisciplinary team approach to CTEPH patient care and share our approach to optimizing care for these patients.     

Cavitating pulmonary infarcts revealing thromboembolic pulmonary hypertension

Several etiologies are involved in the pathogenesis of cavitating pulmonary disease including neoplastic, infectious or inflammatory processes. Another is pulmonary infarction associated with venous thromboembolism. The lung cavities tend to be located peripherally and are the result of pulmonary embolism. We report the case of a woman with chronic thromboembolic pulmonary hypertension (CTEPH), associated with familial thrombophilia, revealed by cavitating pulmonary infarcts. CTEPH is sometimes diagnosed during an episode of recurrent pulmonary embolism following previously unnoticed lesions. Thrombophilias such as isolated elevated factor VIII are risk factors for CTEPH.     

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.     

Controversies, uncertainties and future research on the treatment of chronic thromboembolic pulmonary hypertension

A number of controversies exist regarding the pathophysiology, natural history, diagnosis, and treatment of chronic thromboembolic pulmonary hypertension (CTEPH). Although CTEPH is regarded by many to be a complication of pulmonary embolism (PE) arising subsequent to venous thromboembolism (VTE)-the embolic hypothesis-it has been suggested that PE is rarely the sole cause of CTEPH and that primary arteriopathy with secondary in situ thrombosis may be relevant in the pathogenesis and progression of the disease-the thrombotic hypothesis. A number of lines of evidence support this. Only about half of patients referred with suspected CTEPH have a history of VTE. In addition, data suggest that acute PE is often diagnosed, and possibly misdiagnosed, in patients with preexisting pulmonary artery pathology. There has been much research and debate on the importance of distal arteriopathy in both the initiation and progression of CTEPH. Histopathologic studies have indicated distinct overlap in the microvascular pathology of CTEPH and idiopathic pulmonary arterial hypertension (IPAH), and it has been queried whether class IV CTEPH (increased pulmonary vascular resistance due to distal arteriopathy in the absence of central organized thrombi) and IPAH represent extremes of a disease continuum. Together, these issues may impact on effective diagnosis, preoperative screening criteria for pulmonary endarterectomy surgery, and the likelihood of persistent pulmonary hypertension or even mortality after surgery. They are particularly relevant when considering the possible future use of medical therapies in long-term disease management.