Author/Authors :
Maron، نويسنده , , Barry J. and Roberts، نويسنده , , William C. and Ho، نويسنده , , Carolyn Y. and Kitner، نويسنده , , Carrie and Haas، نويسنده , , Tammy S. and Wright، نويسنده , , Gregory B. and Moazami، نويسنده , , Nader and Feldman، نويسنده , , David S.، نويسنده ,
Abstract :
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.
