摘要
背景:马方综合征心血管病变的主要特征为近端主动脉的进行性扩张。目前尚不清楚此类患者是否会出现左室功能障碍。目的:联合应用磁共振成像(MRI)和组织多普勒成像(TDI)技术,评价无明显心脏瓣膜病的马方综合征患者的左室功能。方法和结果:研究共纳入26例无明显心脏瓣膜病的马方综合征患者(平均年龄32.0±10.9岁,男性12例),同时选取26例年龄和性别均匹配的对象作为对照。
Background: Cardiovascular involvement in Marfan syndrome is mainly characterized by progressive dilatation of the proximal aorta. Whether left ventricular dysfunction is present in these patients is not clear at present. Objectives: Assess left ventricular function in patients with Marfan syndrome, free of significant valvular heart disease, using a combination of MRI and Tissue Doppler imaging(TDI). Methods and results: A total of 26 Marfan patients(mean age=32.0±10.9, 12 men) without significant valvular heart disease, and 26 age-and sex-matched controls were studied. Left ventricular volumes and ejection fraction were measured with magnetic resonance imaging. Systolic and diastolic function parameters were assessed using conventional echocardiography and TDI. When compared to controls, Marfan patients showed impairment of left ventricular contractile function as expressed by a reduced ejection fraction(53.5±9.0%vs. 59.6±6.7%, p=0.009), an increased end-systolic volume(36.0±9.5 vs. 29.5±6.7 ml/m2, p=0.007), and reduced peak systolic velocities at the basal septal and lateral myocardial wall(5.2±1.4 vs. 6.4±1.3 cm/s, p=0.003 and 6.0±2.2 vs. 7.5±2.3 cm/s, p=0.03, respectively). Diastolic function was impaired with an increased deceleration time of the E wave(171±41 ms vs. 141±36 ms, p=0.006). Peak early diastolic velocity at the mitral valve annulus was significantly lower(9.6±2.4 cm/s vs. 11.9±3.3 cm/s, p=0.006). Conclusion: These data provide evidence for mild, but significant impairment of left ventricular systolic and diastolic function in Marfan patients, not related to valvular heart disease.
