Document Detail

Extracardiac medical and neuromuscular implications in restrictive cardiomyopathy.
MedLine Citation:
PMID:  17680617     Owner:  NLM     Status:  MEDLINE    
Restrictive cardiomyopathy (RCMP) is characterized by restrictive filling and reduced diastolic volume of either or both ventricles with normal or near-normal systolic function and wall thickness. It may occur idiopathically or as a cardiac manifestation of systemic diseases such as scleroderma, amyloidosis, Churg-Strauss syndrome, cystinosis, sarcoidosis, lymphoma, Gaucher's disease, hemochromatosis, Fabry's disease, pseudoxanthoma elasticum, hypereosinophilic syndrome, carcinoid, Noonan's syndrome, reactive arthritis, or Werner's syndrome and various neuromuscular disorders. Whereas in idiopathic RCMP the therapeutic options are only treatment of cardiac congestion, in cases with an underlying disorder, a causal therapy may be available. Patients with RCMP should be investigated as soon as the cardiac diagnosis is established for extracardiac diseases to detect a possibly treatable cause of RCMP before the disease becomes intractable. These investigations include a diligent clinical history and examination, blood tests, and ophthalmologic, otologic, dermatologic, gastroenterologic, nephrologic, hematologic, and neurologic examinations. If extracardiac examinations do not reveal a plausible cause for RCMP, endomyocardial biopsy is indicated.
Claudia Stöllberger; Josef Finsterer
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Clinical cardiology     Volume:  30     ISSN:  0160-9289     ISO Abbreviation:  Clin Cardiol     Publication Date:  2007 Aug 
Date Detail:
Created Date:  2007-08-13     Completed Date:  2008-03-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7903272     Medline TA:  Clin Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  375-80     Citation Subset:  IM    
Copyright Information:
(c) 2007 Wiley Periodicals, Inc.
Medizinische Abteilung, Krankenanstalt Rudolfstiftung, Vienna, Austria.
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MeSH Terms
Biological Markers / blood
Cardiomyopathy, Restrictive / complications*,  diagnosis,  genetics,  physiopathology*,  therapy
Endocardium / pathology
Genetic Predisposition to Disease
Heart Catheterization
Myocardium / pathology
Neuromuscular Diseases / etiology*
Stroke Volume
Ventricular Pressure
Reg. No./Substance:
0/Biological Markers

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