Objective: To investigate the peripheral small airway dysfunction differences between idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Methods: Impulse oscillmetory system testing (IOS) and pulmonary function testing (PFT) were performed in IPAH and CTEPH patients and 30 healthy control group. We also carried out a subgroup analysis depending on their medical history of airway diseases. Results: We included 42 IPAH and 47 CTEPH patients (with or without airways disease: 8 vs. 34 and 17 vs. 34, respectively). Compared with CTEPH patients, IPAH patients were younger but had more serious pulmonary vessel resistance and mean pulmonary arterial resistance. Compared with IPAH patients, CTEPH patients had significant impaired peripheral small airway dysfunction with decreased of MEF(50) (% pred), MMEF(75/25) evaluated by PFT and R5-R20, Δ R5-R20 and AX measured by IOS [10.6(2.0, 33.0) vs. 2.5(-5.0, 16.5); 22.1(14.0, 32.6) vs. 15.5 (7.0, 23.2); 7.64(4, 18.6) vs. 6(3, 11) respectively, all P<0.05]. Subgroup analysis revealed there were no significant peripheral small dysfunction differences in IPAH patients with or without airway diseases. CTEPH patients had a higher proportion of airway diseases and more serious peripheral dysfunction than IPAH patients with airway diseases. Compared with control healthy group, peripheral airway dysfunction was more obvious even in IPAH and CTEPH patients without airway diseases. Conclusion: Compared with IPAH, CTEPH patients were older, but had better hemodynamics and a higher proportion of airway diseases. The peripheral airway dysfunction were more serious in CTEPH patients without airway diseases than IPAH patients without airway diseases and healthy controls group.
目的： 比较特发性肺动脉高压（IPAH）及慢性血栓栓塞性肺动脉高压（CTEPH）患者的小气道功能差异。 方法： 回顾性纳入2015年11月至2017年11月上海市肺科医院肺循环科确诊为肺动脉高压的患者89例，其中IPAH患者42例（IPAH组），其中男18例，女29例，年龄26~73岁，平均（46±16）岁；CTEPH患者47例（CTEPH组），其中男13例，女29例，年龄22~80岁，平均（61±13）岁；同期30名健康体检者作为对照组，其中男12名，女18名，年龄24~59岁，平均（54±6）岁。所有患者均进行常规肺功能及脉冲振荡肺功能（IOS）检查，分析IPAH与CTEPH患者肺功能小气道变化特点，并比较合并其他气道疾病对小气道功能的影响。 结果： 与IPAH组比较，CTEPH组患者小气道功能受累更严重，肺功能呼出50%肺活量时最大呼气流量占预计值%（MEF(50)占预计值%）在IPAH组和CTEPH组分别为（74±22）%和（59±27）%。IOS检查CTEPH组振荡频率为5和20 Hz时气道阻力差值（R(5)-R(20)）为10.6（2.0, 33.0），R(5)-R(20)与振荡频率为20 Hz时的气道阻力比值（ΔR(5)-R(20)）为22.1（14.0, 32.6），电抗面积为7.6（4.0, 18.6），均高于IPAH组[2.5（-5.0, 16.5）、15.5（7.0, 23.2）、6.0（3.0, 11.0），均P<0.05]。IPAH组中合并其他气道病变的患者与不合并气道病变的患者相比，小气道功能无明显统计学差异（P>0.05）；合并气道疾病的CTEPH患者小气道功能比合并气道疾病的IPAH患者更差；不合并气道疾病的CTEPH患者小气道功能比不合并气道疾病的IPAH患者及对照组差。 结论： 与IPAH患者相比，CTEPH患者平均确诊年龄高，确诊时血流动力学参数较好，合并气道疾病比例高，小气道功能受损更明显；不合并气道疾病的CTEPH患者的小气道功能差于不合并气道疾病的IPAH患者及健康人群。.
Keywords: Hemodynamics; Hypertension pulmonary; Respiratory function test.