Chronic thromboembolic pulmonary hypertension (CTEPH) is classified as group 4 pulmonary hypertension, characterized by pulmonary arterial thrombotic occlusion leading to vascular stenosis or obstruction, progressive elevation of pulmonary vascular resistance and pulmonary arterial pressure, ultimately leading to right heart failure and even death. Recent years have seen rapid progress in the diagnostic and therapeutic in CTEPH field. More and more patients with CTEPH have been accurately diagnosed and assessed in time. Nevertheless, there is still a lot of work to do in the popularization of CTEPH diagnostic and therapeutic technique and the building of CTEPH expert center. To better guide clinical practice in our country, Pulmonary Embolism & Pulmonary Vascular Diseases Group of the Chinese Thoracic Society, Pulmonary Embolism & Pulmonary Vascular Disease Working Group of Chinese Association of Chest Physicians, National Cooperation Group on Prevention & Treatment of Pulmonary Embolism & Pulmonary Vascular Disease, National Expert Panel on the Development of a Standardized Framework for Pulmonary Arterial Hypertension, convened multidisciplinary experts for deliberation and Delphi expert consensus to develop the \"Guidelines for the Diagnosis and Treatment of Chronic Thromboembolic Pulmonary Hypertension (2024 edition) \". These guidelines systematically evaluate domestic and international evidence-based medical research on CTEPH and propose recommendations tailored to clinical practice in our country. The key areas covered include definitions, epidemiology, pathogenesis, diagnosis and assessment, treatment, and management, with the aim of further standardizing the clinical diagnosis and treatment of CTEPH in our country.
慢性血栓栓塞性肺动脉高压（CTEPH）属于第四大类肺动脉高压（PH），以肺动脉管腔内慢性血栓阻塞与继发肺血管重塑为主要病理特征，继而引起肺动脉管腔狭窄和（或）闭塞，肺血管阻力进行性升高，最终可导致右心衰竭甚至死亡。近年来CTEPH领域诊断及治疗进展迅速，日益增多的患者得到了及时、正确的诊断与病情评估。尽管如此，在CTEPH规范化诊治技术推广、CTEPH中心建设等方面还需进一步加强。为了更好指导我国的临床实践，经过多学科专家研讨和德尔菲专家论证，中华医学会呼吸病学分会肺栓塞与肺血管病学组、中国医师协会呼吸医师分会肺栓塞与肺血管病工作组、全国肺栓塞与肺血管病防治协作组及全国肺动脉高压标准化体系建设项目专家组联合多学科专家制订了《慢性血栓栓塞性肺动脉高压诊断与治疗指南（2024）》。本指南系统评价了国内外CTEPH相关循证医学研究资料，提出符合我国临床实践的推荐意见，主要内容包括：定义、流行病学、发病机制、诊断与评估、治疗与管理，以期进一步规范我国CTEPH的临床诊疗工作。.

Chronic thromboembolic pulmonary hypertension (CTEPH) is classified as group IV pulmonary hypertension, characterized by thrombotic occlusion of the pulmonary arteries leading to vascular stenosis or obstruction, progressive increase in pulmonary vascular resistance and pulmonary arterial pressure, and eventual right heart failure. Unlike other types of pulmonary hypertension, the prognosis of CTEPH can be significantly improved by surgery, vascular intervention, and/or targeted drug therapy. Pulmonary endarterectomy (PEA) is the preferred treatment of choice for CTEPH. However, PEA is an invasive procedure with high operative risks, and is currently only performed in a few centers in China. Balloon pulmonary angioplasty (BPA) is an emerging interventional technique for CTEPH, serving as an alternative for patients who are ineligible for PEA or with residual pulmonary hypertension after PEA. BPA is gaining traction in China, but its widespread adoption is limited due to its complexity, operator skills, and equipment requirements, a lack of standard operating procedures and technical guidance, which limit the further improvement and development of BPA in China. To address this, a multidisciplinary panel of experts was convened to develop the Consensus on the Procedure of Balloon Pulmonary Angioplasty for the Chronic Thromboembolic Pulmonary Hypertension, which fomulates guidelines on BPA procedural qualification, perioperative management, procedural planning, technical approach, and complication prevention, with the aim of providing recommendations and clinical guidance for BPA treatment in CTEPH and standardizing its clinical application in this setting. Summary of recommendations: Recommendation 1: It is recommended that physicians who specialize in pulmonary vascular diseases take the lead in formulating the diagnostic and treatment plans for CTEPH, using a multidisciplinary approach.Recommendation 2: Training in BPA technique is critical; novice operators should undergo standardized operative training with at least 50 procedures under the guidance of experienced physicians before embarking on independent BPA procedures.Recommendation 3: BPA requires catheterization labs, angiography systems, standard vascular interventional devices and consumables, drugs, and emergency equipment.Recommendation 4: Patient selection for BPA should consider cardiac and pulmonary function, coagulation status, and comorbid conditions to determine indications and contraindications, thereby optimizing the timing of the procedure and improving safety.Recommendation 5: In experienced centers, patients deemed likely to benefit from early BPA, based on clinical and imaging features of CTEPH and without elevated D-dimer levels, could bypass standard 3-month anticoagulation therapy.Recommendation 6: BPA is a complex interventional treatment that requires thorough pre-operative assessment and preparation.Recommendation 7: The use of perioperative anticoagulants in BPA requires a comprehensive risk assessment of intraoperative bleeding by the operator for individualized decision making.Recommendation 8: A variety of venous access routes are available for BPA; unless contraindicated, the right femoral vein is usually preferred because of its procedural convenience and reduced radiation exposure.Recommendation 9: For the different types of vascular lesion in CTEPH, treatment of ring-like stenoses, web-like lesions, and subtotal occlusions should be prioritized before addressing complete occlusions and tortuous lesions, in order to reduce complications and improve procedural safety.Recommendation 10: A targeted, incremental balloon dilatation strategy based on vascular lesions is recommended for BPA.Recommendation 11: Intravascular pulmonary artery imaging technologies, such as OCT and IVUS can assist in accurate vessel sizing and confirmation of wire placement in the true vascular lumen. Pressure wires can be used to objectively assess the efficacy of dilatation during BPA.Recommendation 12: Endpoints for BPA treatment should be individually assessed, taking into account improvements in clinical symptoms, hemodynamics, exercise tolerance, and quality of life.Recommendation 13: Post-BPA routine monitoring of vital signs is essential; anticoagulation therapy should be initiated promptly post-procedure in the absence of complications. In cases of intraoperative hemoptysis, postoperative anticoagulation regimen adjustments should be adjusted according to the bleeding severity.Recommendation 14: If reperfusion pulmonary edema occurs during or after BPA, ensure adequate oxygenation, diuresis, and consider non-invasive positive-pressure ventilation if necessary, while severe cases may require early mechanical ventilation assistance or ECMO.Recommendation 15: In cases of intraoperative hemoptysis, temporary balloon occlusion to stop bleeding is recommended, along with protamine to neutralize heparin. Persistent bleeding may warrant the use of gelatin sponges, coil embolization, or covered stent implantation.Recommendation 16: For contrast imaging during BPA, non-ionic, low or iso-osmolar contrast agents are recommended, with hydration status determined by the patient\'s clinical condition, cardiac and renal function, and intraoperative contrast volume used.
慢性血栓栓塞性肺动脉高压（chronic thromboembolic pulmonary hypertension，CTEPH）是以肺动脉血栓机化致血管狭窄或闭塞，肺血管阻力和肺动脉压力进行性升高，最终导致右心功能衰竭为特征的一类疾病，属于肺动脉高压的第4大类。不同于其他类型肺动脉高压，CTEPH可以通过外科手术、血管介入和（或）靶向药物治疗显著改善预后。肺动脉内膜剥脱术（pulmonary endarterectomy，PEA）是CTEPH的首选治疗方法。但其创伤较大、手术风险较高，目前国内仅有少数中心开展。经皮肺动脉球囊成形术（balloon pulmonary angioplasty，BPA）是近年迅速发展的肺动脉介入治疗技术，可作为不适合PEA或PEA术后残余肺动脉高压患者的治疗选择。目前BPA在我国兴起，但由于其操作较复杂，且对术者技术水平和医院设备有一定要求，国内尚缺乏统一的BPA操作规程和技术指导，从而限制国内BPA技术的进一步提升与发展。基于此，为进一步规范和推广BPA技术，经过多学科专家研讨和德尔菲专家论证，工作组牵头制定了《经皮肺动脉球囊成形术治疗慢性血栓栓塞性肺动脉高压操作规程专家共识》，针对BPA操作准入标准、围手术期管理、手术规划、技术方法、并发症防治等诸多方面进行了阐述，以期为BPA治疗CTEPH提供推荐意见和临床指导，规范BPA在CTEPH治疗中的临床应用。 经皮肺动脉球囊成形术治疗慢性血栓栓塞性肺动脉高压操作规程推荐意见： 推荐意见1：建议以从事肺血管疾病诊治工作的医师为主导,多学科协作制定CTEPH的诊治方案。推荐意见2：开展BPA技术培训至关重要，对于初学的操作者，建议在有BPA经验的医师指导下完成至少50例次的标准化操作培训，才可以独立开展BPA术。推荐意见3：开展BPA手术，需要配备导管室、血管造影机、常用血管介入器械和耗材、药品及抢救设备等。推荐意见4：应根据患者心肺功能、凝血功能和合并症情况综合判断，把握BPA适应证和禁忌证，确定最佳的BPA手术时机，提高手术安全性。推荐意见5：对于有经验的中心，如果从临床和影像特征判断为CTEPH，且D-二聚体不高，评估认为能从早期BPA中获益者，可以不经过3个月抗凝治疗。推荐意见6：BPA是一项较复杂的介入手术，需做好充分的术前评估和准备工作。推荐意见7：BPA围手术期抗凝药物的使用需要术者综合评估术中出血风险，个体化决策。推荐意见8：BPA有多种静脉入路选择，为便于操作和减少辐射暴露，在没有禁忌的情况下首选右侧股静脉。推荐意见9：CTEPH血管病变类型较多，为减少并发症，提高手术安全。一般情况下，宜优先处理环形狭窄、网状病变和次全闭塞病变，再处理完全闭塞和迂曲病变。推荐意见10：BPA对靶血管病变采用分次逐级球囊扩张策略。推荐意见11：肺动脉腔内OCT、IVUS成像技术可以帮助精确测定血管直径，并判断导丝是否位于血管真腔内。压力导丝能够准确客观判断扩张效果，在BPA术中运用具有一定价值。推荐意见12：BPA的治疗终点应个体化评估，包括临床症状、肺血流动力学、运动耐量及生活质量的改善。推荐意见13：BPA术后常规行生命体征监护，术中无明显并发症发生者术后尽早启动抗凝治疗。术中发生咯血者，术后根据咯血情况调整抗凝方案。推荐意见14：BPA术中、术后患者出现再灌注肺水肿时，应充分给氧，适度利尿，必要时使用无创正压通气治疗，严重病例需要尽早行有创呼吸机辅助通气或ECMO救治。推荐意见15：BPA术中出现咯血时，建议使用球囊堵塞阻断血流以及鱼精蛋白中和普通肝素。如果持续出血可选择明胶海绵、弹簧圈栓塞或植入覆膜支架。确保咯血停止后方可撤出球囊、导丝和指引导管。推荐意见16：BPA术中造影时，建议使用非离子型低渗或等渗对比剂，根据患者病情、心肾功能及术中对比剂用量决定是否水化。.

Pulmonary embolism (PE) is the third most common acute cardiovascular disease. The risk of PE increases with age and mortality is high. Patients are stratified into hemodynamically stable versus unstable patients, as this has important implications for diagnosis and therapy. Since clinical signs and symptoms of acute PE are nonspecific, the clinical likelihood of PE is estimated to guide diagnostic pathways. D-dimer testing is performed in hemodynamically stable patients with low or intermediate probability of PE and the visualization of thromboembolism and its sequelae is commonly achieved with computed tomography pulmonary angiography (CTPA), supplemented by ultrasound techniques. With confirmed PE, another risk stratification estimates disease severity and defines intensity and setting of the ensuing treatment. The therapeutic spectrum ranges from outpatient treatment with initial oral anticoagulation to thrombolytic or interventional treatment in the intensive care unit or catheterization laboratory. In single cases, even acute surgical thrombectomy is attempted.

Deep vein thrombosis (DVT) and pulmonary embolism (PE) are the most common manifestations of venous thromboembolism (VTE). Most DVTs affect the lower-extremity veins. Since the symptoms of DVT are non-specific, a prompt and standardised diagnostic work-up is essential to minimise the risk of PE in the acute phase and to prevent thrombosis progression, post-thrombotic syndrome and VTE recurrence in the long-term. Only recently, the AWMF S2k guidelines on Diagnostics and Therapy of Venous Thrombosis and Pulmonary Embolism have been revised. In the present article, we summarize current evidence and guideline recommendations focusing on lower-extremity DVT (LEDVT). Depending on whether the diagnostic work-up is performed by a specialist in vascular medicine or by a primary care physician, different diagnostic algorithms are presented that combine clinical probability, D-dimer testing and diagnostic imaging. The diagnosis of ipsilateral recurrent DVT poses a particular challenge and is presented in a separate algorithm. Anticoagulant therapy is an essential part of therapy, with current guidelines clearly favouring regimens based on direct oral anticoagulants over the traditional sequential therapy of parenteral anticoagulants and vitamin K antagonists. For most DVTs, a duration of therapeutic-dose anticoagulation of at least 3 to 6 months is considered sufficient, and this raises the question of the risk of VTE recurrence after discontinuation of anticoagulation and the need for secondary prophylaxis in the long-term. Depending on the circumstances and trigger factors that have contributed to the occurrence of DVT, management strategies are presented that allow decision-making taking into account the individual bleeding risk and patient\'s preferences.

It is an honor and a great pleasure for us to be guest editors for this special issue of Hämostaseologie - Progress in Haemostasis, which addresses important issues surrounding the complex of venous thromboembolism (VTE). In February 2023, the revised guideline on \"Diagnostics and Therapy of Venous Thrombosis and Pulmonary Embolism\" has been published on the website of the Association of the Scientific Medical Societies in Germany (AWMF)1. This guideline was drawn up under the leadership of the German Society of Angiology (DGA), and representatives of 17 scientific societies contributed to its content. As an S2k guideline, its recommendations are consensus based and are the result of a systematic review and evaluation of current evidence and consideration of the benefits and harms of diagnostic and therapeutic options. In this special issue, guideline authors provide a comprehensive overview of selected guideline topics which might be of clinical relevance to our readers and our community of haemostaseologists.

Anticoagulation is the first-line approach in the prevention and treatment of pulmonary embolism. In some instances, however, anticoagulation fails, or cannot be administered due to a high risk of bleeding. Inferior vena cava filters are metal alloy devices that mechanically trap emboli from the deep leg veins halting their transit to the pulmonary circulation, thus providing a mechanical alternative to anticoagulation in such conditions. The Greenfield filter was developed in 1973 and was later perfected to a model that could be inserted percutaneously. Since then, this model has been the reference standard. The current class I indication for this device includes absolute contraindication to anticoagulants in the presence of acute thromboembolism and recurrent thromboembolism despite adequate therapy. Additional indications have been more recently proposed, due to the development of removable filters and of progressively less invasive techniques. Although the use of inferior vena cava filters has solid theoretical advantages, clinical efficacy and adverse event profile are still unclear. This review analyzes the most important studies related to such devices, open issues, and current guideline recommendations.

OBJECTIVE: Optimal thromboprophylaxis in orthopaedic procedures is crucial in an attempt to lower the risk of venous thromboembolism, including deep vein thrombosis and pulmonary embolism. We aim to: 1) identify clinical practice guidelines (CPGs) and recommendations (CPRs) on thromboprophylaxis in adult patients undergoing orthopaedic procedures, and 2) assess the methodological quality and reporting clarity of these guidelines.
METHODS: The study was conducted following the 2020 PRISMA guidelines for a systematic review and has been registered on the international prospective register of systematic reviews (PROSPERO) under the registration number (CRD42023406988). An electronic search was conducted using Medline, Embase, Cochrane, Web of Science, Google Scholar and medRxiv. The search terms used were \"\"adults\", \"orthopedic surgery\", \"orthopedic surgeries\", \"orthopedic surgical procedure\", \"orthopedic surgical procedures\" \"english language\", \"venous thromboembolism\", in all possible combinations (January 2013 to March 2023). The eligible studies were evaluated by four blind raters, employing the Appraisal of Guidelines for Research & Evaluation II (AGREE-II) analysis tool.
RESULTS: The literature research resulted in 931 studies. Finally, a total of 16 sets of guidelines were included in the current analysis. There were 8 national and 8 international CPGs. Eight CPGs made specific recommendations for orthopaedic surgery and referred mostly to joints; one guideline focused on pelvi-acetabular trauma, while the rest were more inclusive and non-specific. Four guidelines, one from the American Society of Hematology (ASH), two from the United Kingdom (UK) and one from India were found to have the highest methodological quality and reporting clarity according to the AGREE-II tool. Inter-rater agreement was very good with a mean Cohens Kappa 0.962 (95 % CI, 0.895-0.986) in the current analysis. So, the reliability of the measurements can be interpreted as good to excellent.
CONCLUSIONS: Optimal thromboprophylaxis in orthopaedic procedures is crucial. The available guidelines were found to be mostly of high methodological quality and inter-rater agreement was very good, according to our study.

In survivors of acute pulmonary embolism (PE), the post-PE syndrome (PPES) may occur. In PPES, patients typically present with persisting or progressive dyspnea on exertion despite 3 months of therapeutic anticoagulation. Therefore, a structured follow-up is warranted to identify patients with chronic thromboembolic pulmonary disease (CTEPD) with normal pulmonary pressure or chronic thromboembolic pulmonary hypertension (CTEPH). Both are currently understood as a dual vasculopathy, that is, secondary arterio- and arteriolopathy, affecting the large and medium-sized pulmonary arteries as well as the peripheral vessels (diameter < 50 µm). The follow-up algorithm after acute PE commences with identification of clinical symptoms and risk factors for CTEPH. If indicated, a stepwise performance of echocardiography, ventilation-perfusion scan (or alternative imaging), N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level, cardiopulmonary exercise testing, and pulmonary artery catheterization with angiography should follow. CTEPH patients should be treated in a multidisciplinary center with adequate experience in the complex therapeutic options, comprising pulmonary endarterectomy, balloon pulmonary angioplasty, and pharmacological interventions.

暂无摘要。

Acute pulmonary embolism (PE) remains a significant cause of morbidity and requires prompt diagnosis and management. The prognosis of affected patients depends on the clinical severity. Therefore, risk stratification is imperative for therapeutic decision-making. Patients with high-risk PE need intensive care. These include patients who have successfully survived resuscitation, with obstructive shock or persistent haemodynamic instability. Bedside diagnostics by means of sonographic procedures are of outstanding importance in this high-risk population. In addition to the treatment of hypoxaemia with noninvasive and invasive techniques, the focus is on drug-based haemodynamic stabilisation and usually requires the elimination or reduction of pulmonary vascular thrombotic obstruction by thrombolysis. In the event of a contraindication to thrombolysis or failure of thrombolysis, various catheter-based procedures for thrombus extraction and local thrombolysis are available today and represent an increasing alternative to surgical embolectomy. Mechanical circulatory support systems can bridge the gap between circulatory arrest or refractory shock and definitive stabilisation but are reserved for centres with the appropriate expertise. Therapeutic strategies for patients with intermediate- to high-risk PE in terms of reduced-dose thrombolytic therapy or catheter-based procedures need to be further evaluated in prospective clinical trials.