60例肺动脉高压患者临床特点及随访分析

Clinical Features and Follow-up Results of 60 Patients with Pulmonary Artery Hypertension

目的分析肺动脉高压(PAH)患者临床特点和随访结果。方法回顾性分析2007年11月至2009年11月在天津医科大学总医院心内科住院并诊断为PAH的60例患者,分析其临床特点,病因构成并进行随访。结果 60例患者中结缔组织病(CTD)相关性PAH共28例(46.7%)。其他疾病相关性PAH共32例(53.3%)。至随访时60例患者中死亡6例,其中3例为CTD相关性PAH。失访6例,平均随访时间为(11.8±8.1)个月。根据病因不同将随访资料完整的患者分为CTD相关PAH组(n=23)及非CTD相关PAH组(n=25),比较基线资料,CTD相关PAH患者6分钟步行试验的距离(6MWD)低于非CTD组(P<0.05),脑钠肽(BNP)、Borg呼吸困难评分、心功能分级、肺动脉收缩压(PASP)均高于非CTD组(P<0.05),加用伊洛前列环素或西地那非治疗人数(n=20)多于非CTD相关PAH患者(n=6)。两组患者随访时病情均有缓解,6MWD增加(P<0.05),Borg呼吸困难评分、PASP、BNP、心功能分级均下降(P<0.05)。结论 PAH患者病因复杂,CTD相关PAH发病率高,一旦合并PAH,病情往往较重,预后较差,多需加用靶向药物强化治疗,在临床诊疗过程中需引起重视。

Objective To analyze the clinical features of patients with pulmonary artery hypertension （PAH） and the results of the following-up. Methods Clinical feature of 60 PAH patients who were diagnosed as PAH from Dec 2007 to Dec 2009 were reviewed retrospectively and those patients were followed up. Results Among the 60 patients, there were 28 patients （46.7%） suffered from Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases （PAH-CTD） and 32 patients （53.3%） suffered from Pulmonary Arterial Hypertension Associated with Non-Connective Tissue Diseases （PAH-Non-CTD）, but after （11.8 ±8.1） months follow-up, 6 patients （3 patients were CTD） died and 6 patients lost＇contact during following up. Those remaining patients were divided into two groups based on pathogen, PAH-CTD group （n=23） and PAH-Non-CTD group （n=25）. Before the follow-up, the PAH-CTD patient＇s condition was obvious more critical than the PAH-Non-CTD patients＇ in 6MWD test, BNP, Borg＇s score, heart function degree and PASP, and 20 PAH-CTD patients took PAH targeted drug, while only 6 cases in PAH-Non-CTD group. In the course of follow-up, the condition had relived somewhat in the two group patients, but the former still serious than the latter. Conclusion Many complicated reasons can lead to PAH. The patients of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases had higher morbidity and unfavorable prognosis. It is beneficial for PAH-CTD patients to he administrated with targeted drug treatment and follow-up visit.