P845Left ventricular dysfunction in marfan syndrome is related to aortic distensibility?


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Abstract

The cardiovascular involvement in Marfan syndrome (MS) is characterized mainly by dilatation and reduced distensibility of the ascending aorta. Left ventricular (LV) dysfunction has been detected in MS, but the pathophysiology is unclear and a primary cardiomyopathy is suggested. The purpose of this study was to evaluatethe LV function in patients with MS and to assess the relationship with the aortic distensibility, as possible underlying mechanism.Methods: We studied 19 patients with MS (32.0±5.6 years, 13 men) without valvular or hypertensive heart disease and a control group of 16 healthy subjects (31±6.2years, 11 men). All underwent echocardiography and cardiac magnetic resonance (CMR) and the following indexes were analyzed: a) Echo: mitral flow velocities E/A ratio and deceleration of mitral E; E' velocity obtained from the lateral mitral ring; E/E' ratio b) CMR: LV volumes indexed to BSA and ejection fraction (EF); maximal size and distensibility of proximal ascending aorta. Distensibility was calculated from maximal (systolic) and minimum (diastolic) area of maximal proximal ascending aorta (cross-sectional images from SSFP sequence) using the formula=(maximum area-minimum area)/[ minimum area x (systolic BP-diastolicBP)].Results: 9 from 17 patients with MS had increased LV end-diastolic volume and decreased EF(<50%). Compared with the control group, patients with MS showed increased LV end-diastolic volume (85.0±5.2vs 70.1±5.3ml/m2, p=0.007), lower LV EF (54.0±7.1%vs 56.0±5.3%, p=0.02) and higher E/E' value (8.5±1.3 vs. 6.2±0.8, p=0.01) as well as larger dimensions of proximal aorta (39±1.4cm vs 32±0.8,p=0.01) and lower distensibility (3.4±1.9 vs. 4.4±1.7, p=0.03). A correlation was found between the aortic distensibility and the LV EF (r=0.47, p=0.045), but not with the other LV functional indexes.Conclusion: In our population of patients with MS, we found changes in the LV function, which were correlated with aortic distensibility. A further study with a larger population will be conducted in order to confirm these results and identify independent predictors of LV dysfunction

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