特发性肺动脉高压的临床和血液动力学分析

摘要

Objective: To investgate the clinical and hemodynamic characteristics of idiopathic pulmonary arterial hypertension (IPAH). Methods; The clinical data of 27 patients with IPAH admitted to Beijing Shijitan Hospital from March 2008 to March 2011 were retrospectively analyzed. All patients were diagnosed by echocardiography and right heart catheterization, and underwent the classification of heart function and the six-minute walk test. Results; Duration from first symptom to diagnosis was (34. 16 ±39. 85) months. The heart function reached WHO Ⅲ-Ⅳ in 74% of the patients. The ratio of male to female was 1:4. The six-minute walking distance was (363. 04 ± 101. 48) m. Pulmonary mean pressure was (64. 60 ± 16. 28) mmHg ( 1 mm-Hg=0. 133 kPa). Cardiac index was (3. 12 ± 1. 22) L-min-1·m-2. The mean pressure of right atrium was (10.40±4.78) mmHg. Pulmonary vascular resistance was (18.04 ±4.65) Wood units. The level of NT-proBNP was ( 3225. 26 ± 728. 5 ) ng/L. Pulmonary systolic pressure evaluated with echocardiography and right heart catheterization were (88. 00 ±22. 67) mmHg and (94. 89 ±30. 24) mmHg respectively, with a high correlation (r =0.795, P≤0. 01). Condution; The demographic characteristics of our patients were similar to those in registry study of NIH. Most patients had severe symptoms indicating impaired function. The hemodynamic variables in patients with WHO Ⅲ-Ⅳ got worse markedly than those with WHO Ⅰ-Ⅱ. We suggest screening and evaluating IPAH and monitoring its progression with echocardiography, classification of heart function, the six-minute walk test and hemodynamic variables.u0000ables in patient%目的:初步分析特发性肺动脉高压(IPAH)患者的临床特征和血流动力学特点.方法:分析2008年3月至2011年3月,北京世纪坛医院住院的27例IPAH患者的临床资料,所有患者均通过静息状态下超声心动图及右心导管检查确诊,并进行PAH功能分级及6 min步行距离检查.结果:患者首发症状至确诊时间为(34.16 ±39.85)个月,74％ PAH患者确诊时WHO功能分级为Ⅲ-Ⅳ级；男女两性之比1:4,6 min步行距离平均(363.04±101.48)m,肺动脉平均压(64.60±16.28) mmHg(1 mmHg=0.133kPa),心脏指数(3.12±1.22) L·min-1·m-2,右心房平均压(10.40±4.78) mmHg,肺血管阻力(18.04±4.65) Wood单位,N末端脑钠肽前体(NT-proBNP)测定值为(3225.26±728.5) ng/L,超声心动图测得肺动脉收缩压及经右心导管检查测得肺动脉收缩压,分别为(88.00±22.67) mmHg,(94.89±30.24) mmHg,二者相关性(r=0.795,P＜0.01).结论:本组PAH的人口学特点与NIH注册研究接近,大多数患者确诊时已出现功能受损的严重症状,Ⅲ/Ⅳ级患者其血流动力学指标较Ⅰ/Ⅱ级患者明显恶化,建议应用超声心动图检查、心功能分级、6 min步行距离及肺血流动力学参数的变化,来对IPAH进行筛查及病情评估、治疗效果评价及病情恶化的监测,可以做到早期发现、早期干预.