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Onset of Apical Hypertrophic Cardiomyopathy in Adulthood.
MedLine Citation:
PMID:  21958740     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
The development of the hypertrophic cardiomyopathy (HC) phenotype with left ventricular (LV) hypertrophy usually occurs in adolescence, and documentation of patients with later onset of wall thickening during adulthood is rare. We report 4 patients with asymptomatic, nonobstructive HC (3 women and 1 man) who were studied with serial cardiovascular magnetic resonance imaging or echocardiography. In these patients, LV wall thickening, confined to the apex and the contiguous distal portions of the ventricular septum and free wall, appeared in midlife and beyond. These patients were >40, >50, or >70 years old when the hypertrophy became evident. The maximum LV wall thickness was 14 to 25 mm (mean 18), with a "spade" deformity of the distal chamber, associated with a nondilated cavity and normal ejection fraction (65% to 80%), in the absence of mitral valve systolic anterior motion. In each patient, similar electrocardiographic patterns with similar diffuse and marked T-wave inversion (with or without increased precordial voltages) preceded the appearance of the HC phenotype on the imaging studies. In conclusion, the recognition that the onset of LV hypertrophy in HC can be delayed well into adulthood (and even to advanced age) has important implications regarding the clinical screening practices for families, and suggests the potential value of extending prospective serial imaging beyond adolescence in some relatives. Electrocardiographic repolarization abnormalities can predict the future development of apical LV hypertrophy in adults with HC.
Authors:
Barry J Maron; Tammy S Haas; Carrie Kitner; John R Lesser
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-9-27
Journal Detail:
Title:  The American journal of cardiology     Volume:  -     ISSN:  1879-1913     ISO Abbreviation:  -     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-9-30     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0207277     Medline TA:  Am J Cardiol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 Elsevier Inc. All rights reserved.
Affiliation:
Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation at Abbott Northwestern Hospital, Minneapolis, Minnesota.
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