Objective: To investigate the clinical characteristics of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Methods: CTEPH cases consecutively admitted into China-Japan Friendship Hospital from September 2015 to June 2019 were enrolled with prospective data collection. The medical histories, clinical characteristics, laboratory tests, imaging manifestation and hemodynamic parameters were analyzed. Patients were divided into high pulmonary vascular resistance (PVR) group and low PVR group according to the PVR level>1 000 dyn·s·cm(-5) or not, and clinical characteristics were compared between these two groups. Results: In the 148 cases of CTEPH, right heart catheterization was performed in 103 cases with mPAP (45.1±11.0) mmHg and PVR of (992±430) dyn·s·cm(-5). At diagnosis, 88 (59.5%) cases were in WHO functional class Ⅲ and 27 (18.2%) in class Ⅳ. Most common presenting symptoms were dyspnea (147, 99.3%), chest tightness (68, 45.9%), hemoptysis (42, 28.3%), syncope (30, 20.3%), and most common signs were P2 accentuation (95, 64.9%), edema (65, 43.9%), cyanosis (47, 31.8%), systolic murmur (44, 29.7%) and jugular vein distention (35, 23.6%). In 103 cases with right heart catheterization, 52 were in the low PVR group and 51 in high PVR group. Compared to the low PVR group, high PVR group patients had higher WHO functional class and more jugular vein distention (both P<0.05). In all the 148 cases, previous venous thromboembolism (VTE) was confirmed in 105 (70.9%) patients, with a higher prevalence of previous VTE in low PVR group than that in high PVR group (P<0.001). 30 (20.3%) patients had varicose veins of the lower extremities, and 21 (14.2%) had other thrombophilic disorders including antiphospholipid syndrome, protein C and S deficiency and antithrombin Ⅲ deficiency. Conclusions: Dyspnea, P2 accentuation and edema are the most common clinical presentation of CTEPH. Previous history of VTE is common in CTEPH patients with thrombophilia in some cases.
目的： 分析慢性血栓栓塞性肺动脉高压（CTEPH）患者的临床特征。 方法： 对2015年9月至2019年6月中日友好医院确诊的CTEPH患者的人口学信息、病史、临床表现及血流动力学资料进行总结，采用描述性研究方法，分析CTEPH患者临床表现及病史特点，并根据肺血管阻力（PVR）是否>1 000 dyn·s·cm(－5)分为高PVR组和低PVR组，比较不同血流动力学状态患者之间临床特征的差异。 结果： 共纳入148例CTEPH患者，103例完成了右心导管检查，肺动脉平均压（mPAP）为（45.1±11.0）mmHg，PVR为（992±430）dyn·s·cm(－5)。88例（59.5%）确诊时肺动脉高压功能分级为Ⅲ级，27例（18.2%）为Ⅳ级。CTEPH最常见的症状为活动后气短，共147例（99.3%），其次是胸闷68例（45.9%）、咯血42例（28.3%）、晕厥30例（20.3%）。最常见的体征是P2亢进95例（64.9%），其他包括下肢水肿65例（43.9%）、口唇发绀47例（31.8%）、三尖瓣区收缩期杂音44例（29.7%）、颈静脉怒张35例（23.6%）等。103例完成右心导管的患者中，低PVR组共52例，高PVR组51例。与低PVR组相比，高PVR组肺动脉高压功能分级更重，颈静脉怒张发生率高（均P<0.05）。所有148例患者中，105例（70.9%）既往曾诊断静脉血栓栓塞症（VTE），30例（20.3%）存在下肢静脉曲张病史，21例（14.2%）存在其他易栓因素，包括抗磷脂综合征、抗凝血酶缺乏、蛋白C与蛋白S缺乏。与高PVR组比较，低PVR组存在VTE和肺栓塞病史的比例更高（均P<0.001）。 结论： 活动后气短是CTEPH最常见症状，P2亢进、下肢水肿是最常见体征，多数CTEPH患者存在VTE病史，部分患者具有明确易栓因素。.
Keywords: Disease Attributes; Hypertension, pulmonary; Pulmonary embolism; Thromboembolism.