Research Original Investigation Cardiac Abnormalities in Patients With Hutchinson-Gilford Progeria Syndrome pediatric and congenital heart disease. Circ 32. Rivera-Torres J, Calvo CJ, Llach A, et al. Cardiac ventricular structure and function in older Cardiovasc Imaging. 2015;8(2):e002504. electrical defects in progeroid mice and participants in the Age, Gene/Environment Hutchinson-Gilford progeria syndrome patients Susceptibility–Reykjavik Study. Circ Cardiovasc 31. Sado DM, Flett AS, Banypersad SM, et al. with nuclear lamina alterations. Proc Natl Acad Sci U Imaging. 2015;8(4):e003039. Cardiovascular magnetic resonance measurement SA. 2016;113(46):E7250-E7259. of myocardial extracellular volume in health and disease. Heart. 2012;98(19):1436-1441. 33. Bell V, Sigurdsson S, Westenberg JJ, et al. Relations between aortic stiffness and left Invited Commentary Left Ventricular Diastolic Dysfunction in Hutchinson-Gilford Progeria Syndrome Diane Fatkin, MD 8,9 Hutchinson-Gilford progeria syndrome (HGPS) is a prema- dial extracellular matrix. These changes are exacerbated by ture aging disorder that affects approximately 1 in 4 to 8 mil- commonly occurring comorbidities, such as hypertension, dia- 1,2 9 lion newborn infants. Affected individuals present with fail- betes, and obesity. Although many of these factors can be ex- ure to thrive and a progeroid appearance, with progressive pected to be relevant to HGPS, it is noteworthy that diastolic manifestation of short stat-
JAMA Cardiology – American Medical Association
Published: Apr 21, 2018
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