不同类型肺动脉高压临床特点分析

Clinical analysis of pulmonary hypertension

目的研究特发性肺动脉高压(IPAH)、慢性血栓栓塞性肺动脉高压(CTEPH)和结缔组织疾病相关肺动脉高压(CTD-PAH)的临床特点。方法回顾性分析北京协和医院1997年1月至2004年9月诊断为IPAH、CTEPH、CTD-PAH住院患者的临床资料。结果 IPAH、CTEPH、CTD-PAH3组患者女/男的比例分别为14/4、6/10、77/5(P<0.01);平均年龄分别为37岁、51岁、41岁(P=0.022)。出现呼吸困难比例分别为100%、100%、84.1%(P=0.008);发生肢端雷诺征分别为5.56%、0、56.1%(P<0.01)。超声心动图肺动脉收缩压分别为(87.38±21.52)mmHg、(89.63±18.39)mmHg、(69.89±21.07)mmHg(P<0.01);并发间质性肺疾病的比例分别为5.56%、0和35.4%(P<0.01)。结论 IPAH和CTD-PAH患者以青中年女性为主,而CTEPH患者则中年男性多见。IPAH和CTEPH组患者超声心动图肺动脉收缩压高于CTD-PAH组,而CTD-PAH患者则肢端雷诺现象明显,更易并发间质性肺疾病。

Objective To investigate the clinical features of idiopathic pulmonary arterial hypertension （IPAH）, chronic thrombocmbolic pulmonary hypertension （CTEPH） and connective tissue disease-associated pulmonary arterial hypertension （CTD-PAH）. Methods Retrospective analysis was performed in patients with physician-diagnosed IPAH, CTEPH or CTD-PAH admitted to Peking Union Hospital between January 1997 and September 2004. Results The female-to-male ratio was 14/4,6/10 and 77/5 in IPAH, CTEPH and CTD-PAH groups （ P 〈 0. 01 ）, and the mean age was 37,51 and 41 years, respectively （ P = 0. 022 ）. 100% , 100% and 84. 1% of patients in the three groups had dyspnea （ P = 0. 008 ）, and 5.56%, 0 and 56. 1% of them had Reynaud＇s phenomenon （P 〈0. 01 ）. The pulmonary arterial systolic pressure （PASP） measured by echocardiogram was （87.38 ± 21.52 ）, （ 89. 63 ± 18. 39） and （69. 89± 21.07） mmHg（ P 〈 0.01 ）. And the percentage of subjects with comphcating interstitial lung disease was 5.56% ,0 and 35.4% （P 〈 0. 01 ）, respectively. Conclusion Young and middle-aged female subjects were common in IPAH and CTD-PAH groups, while middle-aged male subjects were common in CTEPH group. The level of PASP measured by echocardiogram was higher in IPAH or CTEPH group than that in CTD-PAH group. However, patients with CTD-PAH were more likely to have Raynaud＇s phenomenon and to be complicated with interstitial lung digease.