引用本文:龙愉良,管丽华,周达新.慢性血栓栓塞性肺动脉高压的诊疗进展[J].中国临床新医学,2020,13(9):863-868.
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慢性血栓栓塞性肺动脉高压的诊疗进展
龙愉良,管丽华,周达新
200032 上海,复旦大学附属中山医院心内科,上海市心血管病研究所
摘要:
[摘要] 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。
关键词:  慢性血栓栓塞性肺动脉高压  综述  利奥西呱  肺动脉球囊成形术  肺动脉内皮剥脱
DOI:10.3969/j.issn.1674-3806.2020.09.04
分类号:R 544.1+6
基金项目:
Progress in diagnosis and treatment of chronic thromboembolic pulmonary hypertension
LONG Yu-liang, GUAN Li-hua, ZHOU Da-xin
Department of Cardiology, Zhongshan Hospital Affiliated to Fudan University, Shanghai Institution of Cardiovascular Disease, Shanghai 200032, China
Abstract:
[Abstract] Chronic thromboembolic pulmonary hypertension(CTEPH) belongs to the fourth major classification of pulmonary hypertension(PH), and is the only PH that may be cured at present. Without intervention, the long-term prognosis of CTEPH is poor. Therefore, it is very important to make a clear diagnosis and active treatment. Compared with computed tomography(CT) pulmonary angiography, ventilation-perfusion scintigraphy(V/Q scintigraphy) is highly sensitive in diagnosis of CTEPH. The prognosis of CTEPH is closely related to its treatment decisions: all the patients need lifelong anticoagulation, and riociguat is the only targeted drug currently approved for the treatment of CTEPH. Surgical treatment depends on the CTEPH thromboembolic organization sites: for the thromboembolism at the proximal end of the pulmonary artery, pulmonary thromboendarterectomy is feasible, and the cure rate is high, and the postoperative recovery and the long-term prognosis are better; if the lesion site is in the middle part, balloon pulmonary angioplasty(BPA) can be tried, and the balloon dilatation usually needs to be carried out gradually in stages, and the patient′s activity tolerance is significantly improved after dilatation. In addition, balloon dilatation is also used for residual PH after surgical thromboendarterectomy or PH recurrence. If the lesion is located at the end of pulmonary vessels, conservative treatment with drugs is the only option. Combined therapy with multiple treatment methods may be the development direction of CTEPH treatment in the future.
Key words:  Chronic thromboembolic pulmonary hypertension(CTEPH)  Review  Riociguat  Balloon pulmonary angioplasty(BPA)  Pulmonary thromboendarterectomy(PTE)