Profound Left Ventricular Remodeling Associated With LAMP2 Cardiomyopathy - 11/08/11
Doi : 10.1016/j.amjcard.2010.06.035
Barry J. Maron, MD a, b, ⁎ 
, William C. Roberts, MD c, Carolyn Y. Ho, MD d, Carrie Kitner, RN a, b, Tammy S. Haas, RN a, Gregory B. Wright, MD e, Nader Moazami, MD b, David S. Feldman, MD b
, William C. Roberts, MD c, Carolyn Y. Ho, MD d, Carrie Kitner, RN a, b, Tammy S. Haas, RN a, Gregory B. Wright, MD e, Nader Moazami, MD b, David S. Feldman, MD b a Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota
b Abbott Northwestern Hospital, Minneapolis, Minnesota
c Baylor Cardiovascular Research Institute, Dallas, Texas
d Cardiovascular Division, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
e Children's Heart Clinic, Minneapolis, Minnesota
Corresponding author: Tel: 612-863-3996; fax: 612-863-3875Résumé |
Lysosome-associated membrane protein (LAMP2) cardiomyopathy is an X-linked and highly progressive myocardial storage disorder associated with diminished survival, which clinically resembles sarcomeric hypertrophic cardiomyopathy. As shown here in a young woman, the natural history of LAMP2 may demonstrate an extreme profile of left ventricular remodeling with regression of hypertrophy (i.e. marked wall thinning), chamber dilatation, and severe systolic dysfunction, all of which are associated with widespread transmural scarring.
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| This study was supported in part by a grant from the Hearst Foundations, San Francisco, California. |
© 2010
Publié par Elsevier Masson SAS.
Vol 106 - N° 8
P. 1194-1196 - octobre 2010 Retour au numéro
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