Abstract 19055: Myocardial Deformation Analysis Reveals Early Cardiac Abnormalities in Patients With Hutchinson Gilford Progeria Syndrome
Background: Patients with Hutchinson-Gilford Progeria Syndrome (HGPS) demonstrate accelerated aging with premature cardiovascular morbidity and mortality. While left ventricular ejection fraction (LVEF) has been shown to be normal in the absence of significant valve disease, global and regional myocardial deformation has not been previously examined in this population. We used myocardial deformation imaging to evaluate regional ventricular function in patients with HGPS.
Methods: Transthoracic echocardiography with 2-D speckle tracking was used to prospectively evaluate 56 patients with HGPS at a median age of 10.3 (2.2-19.8) years in comparison with 32 age-matched controls. Left ventricular (LV) longitudinal, circumferential and radial strain and, right ventricular (RV) longitudinal strain were calculated using standard techniques.
Results: Despite a normal LVEF (67±5%), HGPS patients showed lower LV global longitudinal and radial strain compared to controls, while global circumferential strain was preserved (Table). Abnormal LV longitudinal strain was confined to the lateral LV free wall with preserved septal deformation. Within the LV, the mid-septal and mid-lateral segments showed the most prominent abnormalities. Right ventricular global longitudinal strain was also lower in patients compared to controls.
Conclusions: In this largest-to-date cohort of HGPS patients, we found abnormalities in LV and RV deformation compared to controls, despite a normal LVEF. We also found a unique pattern of regional variation in LV deformation. Further research is warranted to evaluate the relationship of these abnormalities to clinical outcomes and to determine if deformation parameters can be useful endpoints in clinical trials in this patient population.
Author Disclosures: F.I. Lunze: None. T. Biering-Sørensen: None. S.M. Hegde: None. S.D. Colan: None. L. Smoot: None. L. Gordon: None. M. Kleinman: None. M. Kieran: None. A. Prakash: None.
- © 2016 by American Heart Association, Inc.