Document Detail


Clinical profiles of hypertrophic cardiomyopathy with apical phenotype--comparison of pure-apical form and distal-dominant form.
MedLine Citation:
PMID:  19838003     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Hypertrophic cardiomyopathy (HCM) with an apical phenotype, in which hypertrophy of the myocardium predominantly involves the apex of the left ventricle, is not uncommon in Japan, but its morphologic variations are not well recognized. The aim of this study was to investigate if these variations have different clinical characteristics although they are still confused to be the same. METHODS AND RESULTS: Patients with the apical phenotype were divided into 2 groups, the "pure-apical" form and the "distal-dominant" form, and their clinical profiles were compared. From the study cohort of 264 patients with HCM, 80 (30%) were classified as having the apical phenotype: 51 with the pure-apical form and 29 with the distal-dominant form. The age at diagnosis was approximately 60 years, and in both groups the majority were male. The distal-dominant group had a significantly larger left atrial diameter (43 vs 39 mm) and higher ratio of proven familial HCM (28 vs 6%), and were more symptomatic (New York Heart Association >or=3) at presentation (17 vs 0%). The event-free rate of cardiovascular events in patients with the distal-dominant form was significantly worse (log-rank P=0.012) than that in patients with the pure-apical form (follow-up period: asymptotically approximately 5 years). CONCLUSIONS: The 2 phenotypes of apical HCM should be recognized and distinguished clinically.
Authors:
Toru Kubo; Hiroaki Kitaoka; Makoto Okawa; Takayoshi Hirota; Eri Hoshikawa; Kayo Hayato; Naohito Yamasaki; Yoshihisa Matsumura; Toshikazu Yabe; Masanori Nishinaga; Jun Takata; Yoshinori L Doi
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-10-17
Journal Detail:
Title:  Circulation journal : official journal of the Japanese Circulation Society     Volume:  73     ISSN:  1347-4820     ISO Abbreviation:  Circ. J.     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2009-11-26     Completed Date:  2010-01-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101137683     Medline TA:  Circ J     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  2330-6     Citation Subset:  IM    
Affiliation:
Department of Medicine and Geriatrics, Kochi Medical School, Nankoku, Japan.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Age of Onset
Aged
Aged, 80 and over
Asian Continental Ancestry Group
Cardiomyopathy, Hypertrophic* / complications,  diagnosis,  ethnology,  mortality,  physiopathology
Cardiomyopathy, Hypertrophic, Familial* / complications,  diagnosis,  ethnology,  mortality,  physiopathology
Child
Disease Progression
Disease-Free Survival
Echocardiography
Electrocardiography
Female
Humans
Japan
Male
Middle Aged
Phenotype
Prognosis
Retrospective Studies
Severity of Illness Index
Time Factors
Young Adult

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