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Clinical Characteristics and Natural History of Hypertrophic Cardiomyopathy With Midventricular Obstruction.
MedLine Citation:
PMID:  23728066     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Background: The prevalence, clinical characteristics and natural history of patients with hypertrophic cardiomyopathy (HCM) and midventricular obstruction (MVO) have not been adequately studied. Methods and Results: A single-center cohort consisting of 423 patients (mean age, 49.3±17.2 years; 66.2% male) was thoroughly followed up for a median of 84 months (7 years; range, 6-480 months). MVO, characterized by the echocardiographic appearance of midventricular muscular apposition with a simultaneous mid-cavitary gradient ≥30mmHg, was identified in 34 patients (8%). Patients with MVO tended to be more symptomatic during their initial evaluation (>90% presented with NYHA class ≥II) compared to the rest of the HCM cohort. Apical aneurysm formation was identified in more than one-fourth of patients with MVO (26.5%), being a characteristic of the group. On multivariate Cox regression hazard analysis, presence of MVO strongly predicted progression to end-stage (burnt out) HCM and related heart failure (HF) deaths (hazard ratio, [HR], 2.62; 95% confidence interval [CI]: 1.2-8.8; P=0.047), as well as sudden death and associated lethal arrhythmic events (HR, 3.3; 95% CI: 1.26-8.85; P=0.016). Conclusions: MVO is a distinct phenotype of HCM associated with unfavorable prognosis in terms of end-stage HCM, sudden death and lethal arrhythmic events. The high adverse outcome rate necessitates early recognition of MVO and appropriate therapeutic interventions.
Authors:
Georgios K Efthimiadis; Efstathios D Pagourelias; Despoina Parcharidou; Thomas Gossios; Vasileios Kamperidis; Efstratios K Theofilogiannakos; Zoi Pappa; Soultana Meditskou; Stavros Hadjimiltiades; Christodoulos Pliakos; Haralampos Karvounis; Ioannis H Styliadis
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-5-31
Journal Detail:
Title:  Circulation journal : official journal of the Japanese Circulation Society     Volume:  -     ISSN:  1347-4820     ISO Abbreviation:  Circ. J.     Publication Date:  2013 May 
Date Detail:
Created Date:  2013-6-3     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101137683     Medline TA:  Circ J     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital.
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