Impaired Biventricular Deformation in Marfan Syndrome: A Strain and Strain Rate Study in Adult Unoperated Patients

Abstract

Our study showed a uniform reduction in biventricular deformation in MFS. These findings suggest that assessment of myocardial function using advanced echocardiographic techniques could be more accurate in MFS patient evaluation than conventional echocardiography alone.

Early Detection of Cardiac Dysfunction by Strain and Strain Rate Imaging in Children and Young Adults With Marfan Syndrome

“…To our knowledge, a study showing early detection of subclinical cardiac dysfunction in children and young adults with Marfan syndrome using strain and strain rate imaging has not been reported previously. A recent study by Kiotsekoglou et al 13 showed that strain rate imaging had superior sensitivity and specificity in predicting biventricular deformation in unoperated adult patients with Marfan syndrome. The myocardial dysfunction in Marfan syndrome is presumably due to remodeling of the extracellular matrix and abnormalities in the transforming growth factor β biological pathway due to a deficiency in fibrillin 1.…”

“…Although strain is a measure of tissue deformation and is defined as the change in length normalized to the original length, the strain rate is the rate at which this change occurs 12 . It has been reported that strain and strain rate imaging provide a more sensitive measure of ventricular dysfunction in adults with Marfan syndrome 13 . However, as yet there are no studies evaluating the role of this technique in younger patients to determine how early this primary cardiomyopathy can manifest.…”

Effect of personalized external aortic root support on aortic root motion and distension in Marfan syndrome patients

“…None of these unfavorable effects were observed in this study of blinded pre and post PEARS surgery comparisons with substantial duration of follow-up. The observed trend for a mild decrease in MAPSE during follow-up may be related to intrinsic subclinical ventricular dysfunction often seen in Marfan syndrome patients [31]. An alternative explanation to this finding could be decreased aortic distensibility (increased stiffness) leading to increased hemodynamic load on the left ventricle, however no significant change in aortic distensibility after PEARS surgery was seen in this study.…”

NT-proBNP and diastolic left ventricular function in patients with Marfan syndrome

“…Subclinical cardiomyopathy in patients with MFS has been found in several clinical echocardiographic [23], [24], [25] and magnetic resonance and combined imaging studies [3], [6], [26]. Diastolic dysfunction has been described to be impaired in MFS [27], [28] but NT-proBNP measurements in this context have not been published before.…”