Primary impairment of left ventricular function in Marfan syndrome

Abstract

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/m 2, 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.