Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.

Tissue Doppler imaging identifies myocardial dysfunction in adults with marfan syndrome.

Rybczynski M, Koschyk DH, Aydin MA, Robinson PN, Brinken T, Franzen O, Berger J, Hofmann T, Meinertz T, von Kodolitsch Y

Centre of Cardiology and Cardiovascular Surgery, Department of Cardiology and Angiology, Hamburg, Germany.

BACKGROUND: Successful prevention of aortic complications has lead to improved survival of Marfan syndrome (MFS). With increasing age, however, ventricular arrhythmia and heart failure are emerging as life-threatening manifestations of myocardial dysfunction. HYPOTHESIS: We sought to investigate whether echocardiography with tissue Doppler imaging (TDI) identifies myocardial dysfunction in adults with MFS. METHODS: We performed two-dimensional (2-D) and Doppler echocardiography with TDI in 141 individuals with suspected MFS and competent heart valves, including 28 persons with MFS who had not undergone surgery and 86 healthy controls without inherited connective tissue disorders. RESULTS: Demographic profile, 2-D, mitral and pulmonary venous flow indices, and left ventricular ejection fractions were similar in both groups. Conversely, isovolumic relaxation time (p < 0.001) and deceleration time of E velocity (p = 0.005) were longer, and atrial reversal velocities (p = 0.02), and systolic and early diastolic TD velocities were slower in MFS than in controls (p = 0.01). Multiple linear regression analysis excluded association of reduced systolic and early diastolic TD velocities with mitral valve prolapse or other clinical or echocardiographic features of MFS. CONCLUSIONS: Our study identifies reduced systolic and early diastolic TD velocities in adults with MFS. Further studies are mandatory to elucidate whether TD velocities predict arrhythmia and heart failure in MFS. Copyright (c) 2007 Wiley Periodicals, Inc.

Published 5 February 2007 in Clin Cardiol, 30(1): 19-24.
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