Left Ventricular Diastolic Dysfunction in Hutchinson-Gilford Progeria Syndrome Invited Commentary Invited Commentary Left Ventricular Diastolic Dysfunction in Hutchinson-Gilford Progeria Syndrome Diane Fatkin, MD Hutchinson-Gilford progeria syndrome (HGPS) is a prema- study cohort, neither of these conditions showed significant ture aging disorder that affects approximately 1 in 4 to 8 statistical association with diastolic dysfunction. In 1,2 million newborn infants. Affected individuals present addition, patients with HGPS were severely underweight rather with failure to thrive and a than obese. progeroid appearance, with Additional abnormalities at the cellular level that prolong Related article progressive manifestation of active relaxation and reduce compliance of cardiac muscle have 8,9 short stature, bone and joint been associated with age-related cardiac dysfunction. 1,2 abnormalities, and subcutaneous fat loss. This disorder is in- Changes in cardiomyocyte calcium handling associated with variably fatal in the midteenage years owing to myocardial in- ion channel remodeling, calcium leak, and reduced uptake by farction or stroke associated with severe accelerated athero- the sarcoplasmic reticulum can increase diastolic calcium con- 8,9 sclerosis. Because of the extreme rarity of HGPS, relatively little centration and actin-myosin crossbridge activation. These is known about the full spectrum and outcomes of cardiovas- effects are exacerbated by increases
JAMA Cardiology – American Medical Association
Published: Apr 21, 2018
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