艾森曼格综合征的诊疗及随访分析

目的:观察了解艾森曼格综合征(ES)患者的诊治情况。方法:回顾性分析2007年7月至2012年11月期间,入住北京安贞医院小儿心脏科,且均经心导管检查的ES患者69例,男性18例,女性51例,平均年龄为(16.2±8.5)岁,平均随访时间为(2.0±1.1)年。随访期间所有患者存活,无死亡病例。临床诊断包括房间隔缺损4例,室间隔缺损16例,完全型房室间隔缺损3例,动脉导管未闭8例,并存2或3种以上所述心脏畸形27例,复杂心脏畸形11例。结果:69例患者中有54例患者应用肺动脉高压的靶向药物治疗,其中波生坦治疗23例,西地那非治疗26例,以上两种药物的联合治疗5例,无特殊药物治疗15例。应用波生坦治疗后,患者的肺动脉平均压(mPAP)由(74.8±7.6)mmHg(1 mmHg=0.133 kPa)下降至(70.2±7.3)mmHg(P=0.012),肺主动脉压力比Pp/Ps由[(1.029±0.073)降至(0.943±0.088),P=0.021],肺/体循环流量比(Qp/Qs)由[(0.969±0.235)升至(1.107±0.251),P=0.014],Rp/Rs由(1.005±0.297)降至(0.829±0.220),P=0.014,肺小动脉阻力指数(PVRI)由[(21.9±7.9)Wood.m2降至(18.0±7.4)Wood.m2,P=0.06];应用西地那非治疗后,患者的mPAP由[(82.7±7.5)mmHg下降至(75.7±7.8)mmHg,P=0.014],Pp/Ps由[(1.068±0.495)降至(0.997±0.973),P=0.235],Qp/Qs由(0.928±0.290)升至(1.105±0.304),P=0.100,肺/体循环阻力比(Rp/Rs)由(1.129±0.374)降至(0.798±0.155),P=0.036,PVRI由[(25.8±6.1)Wood.m2降至(20.6±4.6)Wood.m2,P=0.039]。SpO2、6 min步行试验距离、超声心动图、BNP等均有不同程度的改善,纽约心脏病学会心功能分级保持稳定,且未发生不良反应。结论:超过78%的ES患者开始应用肺动脉高压靶向药物进行治疗。ES患者应用肺动脉高压的长期靶向药物治疗是安全有效的,且耐受性好。波生坦和西地那非能够显著降低ES患者的mPAP、Rp/Rs等心导管检查血流动力学参数,并改善运动能力和临床症状。

Follow-up study for patients with eisenmenger syndrome

Objective:To observe and study the diagnosis and treatment in patients with Eisenmenger syndrome.Methods:Retrospectively collected data form 69 patients with Eisenmenger syndrome went through cardiac catheterization(18 males and 51 females) were analyzed from July 2007 through November 2012 in our institute.The mean age of the patients was(16.2±8.5) years old(ranged from 1.5 to 53).The mean follow-up period was(2.0±1.1)years(ranged from 0.5 to 5.4 year).The cardio-vascular abnormalities included 4 ASDs,16 VSDs,3 CAVSDs,8 PDAs,27 patients with more than one abnormalities mentioned above,and 11 with complicated congenital heart diseases.Results:Among them,54 received targeted therapy for pulmonary arterial hypertension: 23 patients had Bosentan,26 patients Sildenafil,and 5 patients had both.Bosentan improved mPAP from [(74.8±7.6)mmHg to(70.2±7.3)mmHg,P=0.012],Pp/Ps from [(1.029±0.073) to(0.943±0.088),P=0.021],Qp/Qs from [(0.969±0.235) to(1.107±0.251),P=0.014],Rp/Rs from(1.005±0.297) to(0.829±0.220),P=0.014],PVRI from [(21.9±7.9) to(18.0±7.4) Wood·m2,P=0.06];Sildenafil,improved mPAP from [(82.7±7.5) to(75.7±7.8)mmHg,P=0.014],Pp/Ps from [(1.068±0.495) to(0.997±0.973),P=0.235],Qp/Qs from [(0.928±0.290) to(1.105±0.304),P=0.100},Rp/Rs from [(1.129±0.374) to(0.798±0.155),P=0.036],PVRI from [(25.8±6.1) to(20.6±4.6) Wood·m2,P=0.039].Percutaneous blood oxygen saturation(SpO2%),Six-minute walking test distance(6MWTD),echocardiography and BIVP were improved after targeted therapy,New York Heart Association functional class(NYHA-FC) remained stable.No any adverse reactions in the treatment.Conclusion:Over 78% patients with Eisenmenger syndrome started to use targeted thrapy for pulmonary arterial hypertension.Long-term targeted therapy for pulmonary arterial hypertension in patients with Eisenmenger syndrome was safe,effective and well tolerated.Bosentan and Sildenafil could improve hemodynamic parameters such as mPAP,Rp/Rs,et al,also improve exercise capacity objectively and relief symptoms subjectively.