Document Detail

Clinical profile and outcome of idiopathic restrictive cardiomyopathy.
MedLine Citation:
PMID:  10831523     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Idiopathic restrictive cardiomyopathy is a poorly recognized entity of unknown cause characterized by nondilated, nonhypertrophied ventricles with diastolic dysfunction resulting in dilated atria and variable systolic function. METHODS AND RESULTS: Between 1979 and 1996, 94 patients (61% women) 10 to 90 years old (mean, 64 years) met strict morphological echocardiographic criteria for idiopathic restrictive cardiomyopathy, mainly dilated atria with nonhypertrophied, nondilated ventricles. None had known infiltrative disease, hypertension of >5 years' duration, or cardiac or systemic conditions associated with restrictive filling. Nineteen percent were in NYHA class I, 53% in class II, and 28% in class III or IV. Atrial fibrillation was noted in 74% of patients and systolic dysfunction in 16%. Follow-up (mean, 68 months) was complete for 93 patients (99%). At follow-up, 47 patients (50%) had died, 32 (68%) of cardiovascular causes. Four had heart transplantation. The death rate compared with actuarial statistics was significantly higher than expected (P<0.0001). Kaplan-Meier 5-year survival was 64%, compared with expected survival of 85%. Multivariate analysis using proportional hazards showed that the risk of death approximately doubles with male sex (hazard ratio [HR] = 2.1), left atrial dimension >60 mm (HR = 2.3), age >70 years (HR = 2.0), and each increment of NYHA class (HR = 2.0). CONCLUSIONS: Idiopathic restrictive cardiomyopathy or nondilated, nonhypertrophic ventricles with marked biatrial dilatation, as defined morphologically by echocardiography, affects predominantly elderly patients but can occur in any age group. Patients present with systemic and pulmonary venous congestion and atrial fibrillation and have a poor prognosis, particularly men >70 years old with higher NYHA class and left atrial dimension >60 mm.
N M Ammash; J B Seward; K R Bailey; W D Edwards; A J Tajik
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Circulation     Volume:  101     ISSN:  1524-4539     ISO Abbreviation:  Circulation     Publication Date:  2000 May 
Date Detail:
Created Date:  2000-06-26     Completed Date:  2000-06-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  2490-6     Citation Subset:  AIM; IM    
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, Minn. 55905, USA.
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MeSH Terms
Age Factors
Aged, 80 and over
Atrial Fibrillation / complications
Cardiomyopathy, Restrictive / complications,  pathology,  physiopathology*
Chronic Disease
Dilatation, Pathologic
Follow-Up Studies
Heart Catheterization
Hypertension / complications
Middle Aged
Multivariate Analysis
Radiography, Thoracic
Sex Factors
Survival Rate
Comment In:
Circulation. 2001 Apr 10;103(14):E83   [PMID:  11294820 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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