CAJ | 학술논문

目的探讨我国特发性肺动脉高压( IPAH)患者的临床发病特征.方法对同济大学附属上海市肺科医院心肺血管中心2008年6月至2010年6月连续确诊的161例IPAH患者的临床资料进行回顾性分析研究.结果 IPAH患者的平均诊断年龄为(33±15)岁,以女性为主(70.2％).从出现症状到右心导管确诊的中位时间为12个月；多数患者(56.5％)初诊时心功能已达Ⅲ～Ⅳ级；6 min步行距离平均为(398±108)m；肺功能检查示有7.8％、42.2％和82.2％的患者分别存在轻度阻塞性、限制性通气功能和弥散功能障碍.右心导管检查显示确诊时患者肺动脉平均压[(63 ±17) mm Hg(1 mm Hg=0.133 kPa)]和肺血管阻力指数[(25±12) Wood U/m2]均已显著增高.IPAH患者中急性肺血管扩张试验阳性患者比率为8.7％,其中28.6％为儿童患者.相对阴性患者,急性肺血管扩张试验阳性患者年龄较小,肺循环血液动力学参数受损程度较轻.在急性肺血管扩张试验阴性的患者中,有89％患者接受了至少1种肺动脉高压特异性治疗药物,接受联合药物治疗的比例为27％.结论我国IPAH多累及中青年女性,早期诊断困难,确诊时心功能、运动耐量和血液动力学指标往往已显著受损；绝大多数IPAH患者接受肺动脉高压特异性治疗,但相当比例患者治疗强度不够.
목적 탐토아국특발성폐동맥고압( IPAH)환자적림상발병특정.방법 대동제대학부속상해시폐과의원심폐혈관중심2008년6월지2010년6월련속학진적161례IPAH환자적림상자료진행회고성분석연구.결과 IPAH환자적평균진단년령위(33±15)세,이녀성위주(70.2％).종출현증상도우심도관학진적중위시간위12개월；다수환자(56.5％)초진시심공능이체Ⅲ～Ⅳ급；6 min보행거리평균위(398±108)m；폐공능검사시유7.8％、42.2％화82.2％적환자분별존재경도조새성、한제성통기공능화미산공능장애.우심도관검사현시학진시환자폐동맥평균압[(63 ±17) mm Hg(1 mm Hg=0.133 kPa)]화폐혈관조력지수[(25±12) Wood U/m2]균이현저증고.IPAH환자중급성폐혈관확장시험양성환자비솔위8.7％,기중28.6％위인동환자.상대음성환자,급성폐혈관확장시험양성환자년령교소,폐순배혈액동역학삼수수손정도교경.재급성폐혈관확장시험음성적환자중,유89％환자접수료지소1충폐동맥고압특이성치료약물,접수연합약물치료적비례위27％.결론 아국IPAH다루급중청년녀성,조기진단곤난,학진시심공능、운동내량화혈액동역학지표왕왕이현저수손；절대다수IPAH환자접수폐동맥고압특이성치료,단상당비례환자치료강도불구.
Objective To investigate the clinical characteristics of patients with idiopathic pulmonary arterial hypertension (IPAH) in China.Methods A total of 161 patients diagnosed as IPAH in Shanghai Pulmonary Hospital from June 2008 to June 2010 were retrospective analyzed.Results The mean diagnostic age was (33 ± 15 ) years old and 70.2％ patients were female. The median duration from symptoms onset to diagnostic right heart catheterization was 12 months.Incidence of NYHA class Ⅲ to Ⅳ was 56.5％ at diagnosis and the mean six minutes walk distance was limited to (398 ± 108) meters.Incidence of mild obstructive,restrictive and diffusing impairment in pulmonary function test was 7.8％,42.2％ and 82.2％ patients with IPAH,respectively. Right heart catheterization demonstrated severe elevated mean pulmonary arterial pressure [ ( 63 ± 17 ) mm Hg ( 1 mm Hg =0.133 kPa) ] and pulmonary vascular resistance index [ ( 25 ± 12 ) Wood U/m2 ] in this patient cohort.The response rate of acute pulmonary vasoreactivity testing was 8.7％ in this cohort. Compared with non-responders,responders to acute pulmonary vasoreactivity testing were younger and with less severe pulmonary hypertension. Among non-responders,89％ patients were treated by one specific anti-pulmonary arterial hypertension drug and 27％ patients received combined anti-pulmonary arterial hypertension medications.Conclusions Young female was predominantly involved in patients with IPAH in China.The diagnosis of IPAH is often made at advanced disease stage and majority patients with IPAH received specific anti-pulmonary arterial hypertension therapies in this patient cohort.