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

Abstract

Strain and strain rate imaging have been reported to detect cardiac dysfunction more accurately than conventional methods in adults with Marfan syndrome, but their utility has not been proven in younger patients. We sought to determine whether strain and strain rate imaging would allow early detection of cardiac dysfunction in children and young adults with Marfan syndrome. Unoperated patients (<30 years) with Marfan syndrome and healthy control participants were prospectively enrolled. Patients with greater than mild mitral or aortic insufficiency were excluded. Left ventricular systolic function was assessed by standard M-mode imaging. The strain and strain rate values were obtained from apical 4-chamber (longitudinal) and parasternal short-axis views at basal and midventricular levels (radial and circumferential). Data from the two groups were compared by a t test. Sixteen patients with Marfan syndrome (mean age ± SD, 14.4 ± 6.4 years; range, 5.8-28.9 years) and 26 controls (mean age, 12.4 + 4.4 year; range, 4.1-18.1 years) were enrolled. Demographics and left ventricular end-diastolic dimensions were similar between the patients with Marfan syndrome and the controls. The M-mode-derived shortening fraction was significantly lower in the patients with Marfan syndrome compared to the controls, even though the values were within normal limits. The patients with Marfan syndrome had lower regional radial and circumferential strain rates, but there were no significant difference in strain between the groups. Strain rate imaging may be useful in detection of subclinical changes in cardiac function in patients with Marfan syndrome when conventional echocardiographic parameters are within normal limits. These findings may be clinically important and warrant closer follow-up of these patients to monitor for cardiac dysfunction.