Early impairment of left ventricular long-axis systolic function demonstrated by reduced atrioventricular plane displacement in patients with Marfan syndrome

Kiotsekoglou, Anatoli; Bajpai, Abhay; Bijnens, Bart; Kapetanakis, Venedictos; Athanassopoulos, George; Moggridge, James C; Mullen, Mike J; Nassiri, Dariush K; Camm, John; Sutherland, George R; Child, Anne H

doi:10.1093/ejechocard/jen003

Early impairment of left ventricular long-axis systolic function demonstrated by reduced atrioventricular plane displacement in patients with Marfan syndrome

Author:

Kiotsekoglou, Anatoli

Bajpai, Abhay ; Bijnens, Bart ; Kapetanakis, Venedictos ; Athanassopoulos, George ; Moggridge, James C ; Mullen, Mike J ; Nassiri, Dariush K ; Camm, John ; Sutherland, George R ; Child, Anne H

Keywords:

Adolescent, Adult, Aged, Atrioventricular Node, Female, Heart Ventricles, Humans, Male, Marfan Syndrome, Middle Aged, Stroke Volume, Systole, Time Factors, Young Adult, Science & Technology, Life Sciences & Biomedicine, Cardiac & Cardiovascular Systems, Cardiovascular System & Cardiology, Marfan syndrome, left ventricular systolic function, atrioventricular plane displacement, long-axis function, tissue Doppler imaging, CHAMBER QUANTIFICATION, DILATED CARDIOMYOPATHY, EXTRACELLULAR-MATRIX, STANDARDS COMMITTEE, OF-ECHOCARDIOGRAPHY, AORTIC-VALVE, RECOMMENDATIONS, DISEASE, DIMENSIONS, FIBRILLIN, Ultrasonography, 1102 Cardiorespiratory Medicine and Haematology, Cardiovascular System & Hematology

Abstract:

AIMS: Marfan syndrome (MFS) is a connective tissue disorder caused by mutations in the fibrillin-1 (FBN1) gene. It has been observed that FBN1 deficient mice have reduced left ventricular (LV) systolic function which is correlated to increased transforming growth factor-beta activity. This study aimed to ascertain LV functional abnormalities in MFS patients using M-mode and tissue Doppler imaging (TDI). METHODS AND RESULTS: In 66 (15-58 years) MFS patients and 61 normal controls, ejection fraction (EF) was evaluated by Simpson's biplane method. Atrioventricular plane displacement (AVPD) obtained from five mitral annular regions was also assessed using M-mode and TDI techniques. To overcome limitations associated with conventional M-mode echocardiography, anatomical and colour anatomical M-mode were also utilized. Ejection fraction was significantly reduced in MFS patients when compared to controls (66.3 +/- 0.74 vs. 71.9 +/- 0.56, P < 0.001), although it was within the normal range. M-mode and TDI AVPD measurements obtained from lateral, septal, inferior, anterior and posterior mitral annular regions were also significantly reduced in MFS patients in comparison to controls (P <0.001, for all measurements). CONCLUSION: Left ventricular long-axis systolic function is significantly reduced in MFS patients. This data suggests that LV function should be monitored in MFS and appropriate treatment applied if necessary.