| Cardiovascular manifestations in Fabry disease: a clinical and echocardiographic study. | |
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MedLine Citation:
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PMID: 17452128 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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This study reviews the clinical and echocardiographic findings in a cohort of Fabry patients (n=12) and compares echocardiographic findings to normal controls. Almost all patients had extracardiac manifestations. Five out of 12 patients had cardiovascular symptoms. Nine out of 12 patients had left ventricular hypertrophy (LVH) on the electrocardiogram (ECG) and one patient had short PR interval. Three patients had epicardial coronary disease. Four patients had 'rat-tail' appearance on left ventriculogram. Six patients who had myocardial biopsy showed extensive vacuolation of the myocytes on light microscopy and concentric, myelinoid lamellar cytoplasmic inclusion bodies on electron microscopy. On echocardiography, LV mass was significantly increased in the Fabry group compared to normal controls. Traditional parameters of diastolic function including peak E velocity, peak A velocity and deceleration time were no different to normal controls. The IVRT was significantly prolonged in the Fabry subjects. The PV atrial reversal duration exceeded that of mitral A wave duration in the Fabry group. The septal E' velocity with Doppler tissue imaging (DTI) was significantly lower in the Fabry group than the normal controls. Fabry disease should be considered in the differential diagnosis in patients with unexplained LVH and late onset hypertrophic cardiomyopathy. Extracardiac manifestations are common. |
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Authors:
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Norman Sadick; Liza Thomas |
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Publication Detail:
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Type: Journal Article Date: 2007-04-23 |
Journal Detail:
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Title: Heart, lung & circulation Volume: 16 ISSN: 1443-9506 ISO Abbreviation: Heart Lung Circ Publication Date: 2007 Jun |
Date Detail:
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Created Date: 2007-05-23 Completed Date: 2007-08-10 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 100963739 Medline TA: Heart Lung Circ Country: Australia |
Other Details:
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Languages: eng Pagination: 200-6 Citation Subset: IM |
Affiliation:
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Westmead Hospital, Westmead, NSW 2145, Australia. normansadick@swiftdsl.com.au |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Cohort Studies Echocardiography Fabry Disease / complications*, physiopathology* Female Heart / physiopathology Heart Diseases / etiology*, pathology, physiopathology* Humans Male Middle Aged Myocardium / pathology, ultrastructure |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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