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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 and follow-up results of patients with pulmonary hypertension (PAH). Methods A retrospective analysis of 60 patients with PAH diagnosed as PAH from Department of Cardiology, Tianjin Medical University General Hospital from November 2007 to November 2009 was retrospectively analyzed. The clinical features, etiology and follow-up were analyzed. Results 60 cases of CTD-related PAH in 28 cases (46.7%). Other disease-related PAH in 32 cases (53.3%). Six of 60 patients died at follow-up, of which 3 were CTD-related PAH. Six patients were lost to follow-up, the average follow-up time was (11.8 ± 8.1) months. Patients with complete follow-up data were divided into CTD-related PAH group (n = 23) and non-CTD-related PAH group (n = 25). Baseline data were compared for 6-min walking distance The levels of BNP, Borg dyspnea, heart function, and pulmonary artery systolic pressure (PASP) in non-CTD group were significantly higher than those in non-CTD group (P <0.05) The number of patients treated with either plain or sildenafil (n = 20) was higher than those without PAD (n = 6). Both groups were relieved at 6MWD (P <0.05), Borg dyspnea score, PASP, BNP, and heart function grade decreased (P <0.05). Conclusions The etiology of PAH is complex and the incidence of CTD-related PAH is high. Once the PAH is combined, the PAH is often severe and the prognosis is poor. Therefore, it is necessary to add the targeted drug to strengthen the PAH treatment, which needs attention in the clinical diagnosis and treatment.