American Journal of Cardiology
Volume 106, Issue 8 , Pages 1194-1196, 15 October 2010

Profound Left Ventricular Remodeling Associated With LAMP2 Cardiomyopathy

  • Barry J. Maron, MD

      Affiliations

    • Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota
    • Abbott Northwestern Hospital, Minneapolis, Minnesota
    • Corresponding Author InformationCorresponding author: Tel: 612-863-3996; fax: 612-863-3875
    • ,
  • William C. Roberts, MD

      Affiliations

    • Baylor Cardiovascular Research Institute, Dallas, Texas
    • ,
  • Carolyn Y. Ho, MD

      Affiliations

    • Cardiovascular Division, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
    • ,
  • Carrie Kitner, RN

      Affiliations

    • Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota
    • Abbott Northwestern Hospital, Minneapolis, Minnesota
    • ,
  • Tammy S. Haas, RN

      Affiliations

    • Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota
    • ,
  • Gregory B. Wright, MD

      Affiliations

    • Children's Heart Clinic, Minneapolis, Minnesota
    • ,
  • Nader Moazami, MD

      Affiliations

    • Abbott Northwestern Hospital, Minneapolis, Minnesota
    • ,
  • David S. Feldman, MD

      Affiliations

    • Abbott Northwestern Hospital, Minneapolis, Minnesota

Received 12 March 2010; received in revised form 20 April 2010; accepted 20 April 2010. published online 01 September 2010.

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.

PII: S0002-9149(10)01224-5

doi:10.1016/j.amjcard.2010.06.035

American Journal of Cardiology
Volume 106, Issue 8 , Pages 1194-1196, 15 October 2010

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