成人先天性心脏病合并Eisenmenger综合征患者的临床特征

Clinical features of adult patients with Eisenmenger syndrome associated with different types ofcongenital heart disease

目的了解成人先天性心脏病（CHD）合并Eisenmenger综合征患者的临床特征。方法自2007年5月至2010年10月，在全国31个临床中心入选经超声心动图和右心导管检查确诊的成人CHD合并Eisenmenger综合征患者，收集所有患者的年龄、性别、体质指数（BMI）等人口学指标，症状、体征、世界卫生组织肺动脉高压功能分级（WHO-FC）、6min步行距离等临床指标和血流动力学指标。分析不同类型CHD合并Eisenmenger综合征患者的临床特征和血流动力学的差异。结果共纳入224例患者，其中房间隔缺损67例（29．9％），室间隔缺损104例（46．4％），动脉导管未闭53例（23．7％）；男67例（29．9％），女157例（70．1％）；年龄15～63岁，平均（29．6±9．9）岁；平均BMI（19．9±4．0）kg／m。、6min步行距离（371±75）m。WHO-FCⅡ级158例（70．5％），Ⅲ级64例（28．6％）；173例（77．2％）患者心电图表现出右室肥厚。患者平均右房压（8．9±5．7）mmHg（1mmHg=0．133kPa）、肺动脉平均压（77．2±19．1）mmHg、心指数（3．03±1．35）L·min·m-2、肺血管阻力为（1621±887）dyn·S·cm-5。结论CHD合并Eisenmenger综合征患者以中青年女性多见，室间隔缺损患者所占比例最高。CHD患者肺动脉压力和肺血管阻力升高程度与心功能减退不呈平行关系。

Objective To explore the clinical features and hemodynamies of adult patients with Eisenmenger syndrome in different types of congenital heart diseases （ CHD ） . Methods Patients with Eisenmenger syndrome with different types of CHD diagnosed by right heart catheterization were enrolled from 31 clinical centers in China during the period from May 2007 to October 2010. Age, gender, body mass index （BMI）, symptoms and signs, World Health Organization functional class （WHO-FC） of pulmonary hypertension, six-minute walk distance （6MWD） and hemodynamics were recorded. All the above indices were analyzed and compared. Results A total of 224 patients with Eisenmenger syndrome with 3 kinds of CHD were enrolled, including atrial septal defect （n = 67 ） , ventricular septal defect （n = 104 ） and patent ductus arteriosus （n =53）. Among them, there were 67 males （29.9%） and 157 females （70. 1% ） with a mean age of （29. 6 ± 9. 9） years （ range ： 15 - 63 ）. Mean BMI was （ 19.9± 4.0） kg/m2 and mean 6MWD （371 ±75） m. The majority of patients were in WHO-FC 1/ （n = 158, 70.5%） and m （n =64, 28.6% ）. Electrocardiogram of 77.2% of them indicated hypertrophic right ventricle. Mean right atrial pressure was （8. 9 ±5.7） mm Hg （ 1 mm Hg =0. 133 kPa）, mean pulmonary arterial pressure （mPAP） （77.2 ±19.1） mm Hg, cardiac index （3.03 ± 1.35） L ·min-1 · m-2 and pulmonary vascular resistance （PVR） （1621± 887） dyn · s · cm-5. Conclusions The majority of patients with Eisenmenger syndrome with different types of CHD are young females and ventricular septal defect is the most frequent underlyingcause. The deterioration of heart function in patients with Eisenmenger syndrome is non-parallel to mPAP and PVR in CHD.