CAJ | 학술논문

目的分析华中地区世界卫生组织(WHO)第Ⅰ类肺动脉高压(PAH)患者的临床特征.方法 2009年1月至2013年10月武汉亚洲心脏病医院收治的PAH患者195例,其中先天性心脏病相关肺动脉高压(CHD-PAH) 144例,特发性肺动脉高压(IPAH)51例.对上述患者的临床特征进行回顾性分析.结果患者平均年龄(27.5±13.2)岁(1～ 68岁),其中女性129例(66.2％).初诊时WHO心功能Ⅲ、Ⅳ级占32.3％ (63/195),6 min步行距离平均为(397±74)m.IPAH患者从出现症状至右心导管检查确诊的中位时间为38个月.CHD-PAH患者和IPAH患者右心导管检查的指标肺动脉平均压、肺血管阻力指数、心指数等血液动力学指标差异均无统计学意义.急性肺血管扩张试验阳性0人次.接受肺动脉靶向药物治疗的患者共121例,心功能均得到改善,死亡1例.74例患者仅接受了基础治疗,随访期间死亡5例.结论华中地区的WHO第Ⅰ类PAH以CHD-PAH为主,患者确诊时心功能多较差,血液动力学指标受损严重.大部分患者已经接受了靶向药物治疗,但强度不够.
목적 분석화중지구세계위생조직(WHO)제Ⅰ류폐동맥고압(PAH)환자적림상특정.방법 2009년1월지2013년10월무한아주심장병의원수치적PAH환자195례,기중선천성심장병상관폐동맥고압(CHD-PAH) 144례,특발성폐동맥고압(IPAH)51례.대상술환자적림상특정진행회고성분석.결과 환자평균년령(27.5±13.2)세(1～ 68세),기중녀성129례(66.2％).초진시WHO심공능Ⅲ、Ⅳ급점32.3％ (63/195),6 min보행거리평균위(397±74)m.IPAH환자종출현증상지우심도관검사학진적중위시간위38개월.CHD-PAH환자화IPAH환자우심도관검사적지표폐동맥평균압、폐혈관조력지수、심지수등혈액동역학지표차이균무통계학의의.급성폐혈관확장시험양성0인차.접수폐동맥파향약물치료적환자공121례,심공능균득도개선,사망1례.74례환자부접수료기출치료,수방기간사망5례.결론 화중지구적WHO제Ⅰ류PAH이CHD-PAH위주,환자학진시심공능다교차,혈액동역학지표수손엄중.대부분환자이경접수료파향약물치료,단강도불구.
Objective To analyze the clinical characteristics of WHO Class Ⅰ pulmonary hypertension (PAH) in central China.Methods Data was collected as a part of prospective registry of PAH through Jan.2009 to Oct.2013 in Wuhan Asia Heart Hospital.A total of 195 patients were recruited including 144 cases with congenital heart disease with pulmonary hypertension (CHD-PAH) and 51 cases with idiopathic pulmonary hypertension(IPAH).Results The age of all patients ranged from 1 to 68 years (mean (27.5 ± 13.2) years),129 cases were female(66.2％).WHO Class Ⅰ PAH accounted for 91.1％,CHD-PAH 67.3％,IPAH 23.8％,and other 8.9％.WHO function class Ⅲ/Ⅳ in newly diagnosed PAH accounted for 32.3％,the mean 6MWD was (397 ±74) m.For patients with IPAH,the median time period between onset of symptoms and diagnosis by right heart catheterization was 38 months.The mean pulmonary pressure,pulmonary vascular resistance index,cardiac index of patients with IPAH and CHDPAH were measured by the right heart catheterization and there was no difference between the two groups.Acute pulmonary vasodilator testing was negative in all patients in this cohort.Cardiac function was improved in the 121 cases who received the targeted drug treatment and 1 patient died out of these 121 patients while 5 cases died out of patients receiving conventional therapy.Conclusion In Central China,Class Ⅰ pulmonary hypertension is the most predominant type of PAH,the cardiac function and hemodynamic indexes of these patients were significantly impaired at the time of first PAH diagnosis.Most of the patients accepted targeted drug treatment of pulmonary hypertension,but the drug dose used for the targeted drug treatment is not effective enough in these patients.