The management pattern and outcomes of chronic thromboembolic pulmonary hypertension: rationale and design for a Chinese real-world study.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disorder with substantial morbidity and mortality, also a disease underdiagnosed and undertreated. It is potentially curable by pulmonary endarterectomy (PEA) in patients with surgically accessible thrombi. Balloon pulmonary angioplasty (BPA) and targeted medical therapy are options for patients with distal lesions or persistent/recurrent pulmonary hypertension after PEA. There is an urgent need to increase the awareness of CTEPH. Qualified CTEPH centers are still quite limited. Baseline characteristics, management pattern and clinical outcome of CTEPH in China needs to be reported. The CHinese reAl-world study to iNvestigate the manaGEment pattern and outcomes of chronic thromboembolic pulmonary hypertension (CHANGE) study is designed to provide the multimodality treatment pattern and clinical outcomes of CTEPH in China. Consecutive patients who are ≥ 14 year-old and diagnosed with CTEPH are enrolled. The diagnosis of CTEPH is confirmed in right heart catheterization and imaging examinations. The multimodality therapeutic strategy, which consists of PEA, BPA and targeted medical therapy, is made by a multidisciplinary team. The blood sample and tissue from PEA are stored in the central biobank for further research. The patients receive regular follow-up every 3 or 6 months for at least 3 years. The primary outcomes include all-cause mortality and changes in functional and hemodynamic parameters from baseline. The secondary outcomes include the proportion of patients experiencing lung transplantation, the proportion of patients experiencing heart and lung transplantation, and changes in health-related quality of life. Up to 31 December 2023, the study has enrolled 1500 eligible patients from 18 expert centers. As a real-world study, the CHANGE study is expected to increase our understanding of CTEPH, and to fill the gap between guidelines and the clinical practice in the diagnosis, assessment and treatment of patients with CTEPH. REGISTRATION NUMBER IN CLINICALTRIALS.GOV: NCT05311072.

Zhang S ，Li Y ，Yang Y ，Gong S ，Yang Z ，Hong C ，Cui X ，Wan J ，Ji Y ，Chen H ，Zhu L ，Li C ，Cheng Z ，Zhang Y ，Wang Q ，Luo Q ，Guo L ，Ma G ，Han B ，Liu Z ，Xiong C ，Wang L ，Ji Q ，Li F ，Wang S ，Zhang Z ，Zhang Y ，Wang D ，Gao Q ，Huang Q ，Xie W ，Zhai Z ，Wang C ... -  《BMC Pulmonary Medicine》

[Consensus on the procedure of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension].

Group of Pulmonary Embolism and Pulmonary Vascular Disease, Chinese Association of Chest Physicians ，Working Committee of Pulmonary Embolism and Pulmonary Vascular Disease, Chinese Thoracic Society ，Expert Committee, National Project of Standardized Diagnosis and Treatment of Pulmonary Hypertension ，Expert Committee of Pulmonary Embolism and Pulmonary Vascular Disease, National Center for Respiratory Medicine ... -  《-》

Treatment of chronic thromboembolic pulmonary hypertension in a multidisciplinary team.

Siennicka A ，Darocha S ，Banaszkiewicz M ，Kędzierski P ，Dobosiewicz A ，Błaszczak P ，Peregud-Pogorzelska M ，Kasprzak JD ，Tomaszewski M ，Mroczek E ，Zięba B ，Karasek D ，Ptaszyńska-Kopczyńska K ，Mizia-Stec K ，Mularek-Kubzdela T ，Doboszyńska A ，Lewicka E ，Ruchała M ，Lewandowski M ，Łukasik S ，Chrzanowski Ł ，Zieliński D ，Torbicki A ，Kurzyna M ... -  《-》

Advances in treatment of chronic thromboembolic pulmonary hypertension.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a progressive pulmonary vascular disease which can lead to right heart failure and death, if left untreated. CTEPH is caused by persistent obstruction of large, middle-sized, or distal pulmonary arteries due to limited thromboembolic resolution in the pulmonary vascular arterial tree. Every patient with CTEPH should undergo evaluation for Pulmonary Endarterectomy (PEA) after referral to institutions with an experienced multidisciplinary CTEPH team. Although management of distal thromboembolic lesions with PEA remains a challenge due to their difficult accessibility, limited distal CTEPH is not considered an absolute contraindication for PEA, as more expertise surgical teams operate on them successfully. Furthermore, in up to 30-50% of patients who undergo PEA, curative treatment is not achieved due to incomplete thrombi removal or extensive pulmonary microvascular disease. Medical therapies that target the underlying pulmonary microvascular disease can offer symptomatic and hemodynamic benefits, although they do not deal with the core mechanism of the disease which is the removal of thromboembolic material from pulmonary vasculature. Recent research has provided evidence suggesting balloon pulmonary angioplasty (BPA) is a reasonable treatment option for inoperable CTEPH and recurrent/persistent pulmonary hypertension after PEA. Advancements in diagnostic modalities and refinements of BPA technique have decreased the complication rate and increased its beneficial effects in hemodynamics, symptoms, right ventricular function and long-term survival. Ongoing trials and future prospective cohorts will provide evidence regarding the optimal selection of patients and lesions prone to BPA treatment along with hybrid therapeutic strategies combining pharmacological therapy, PEA and BPA, which can potentially change the standard of care in CTEPH.

Tzoumas A ，Peppas S ，Sagris M ，Papanastasiou CA ，Barakakis PA ，Bakoyiannis C ，Taleb A ，Kokkinidis DG ，Giannakoulas G ... -  《-》

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.

Tanabe N ，Sugiura T ，Tatsumi K 《-》