| The right ventricle in Fabry disease. | |
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MedLine Citation:
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PMID: 15895706 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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AIM: Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease. METHODS: Echocardiographic examination of the right and left ventricle was carried out in 129 patients (80 women and 49 men) with Fabry disease. RESULTS: RV hypertrophy was present in 46 patients (35.7%). Of these patients, 13 showed signs of severely depressed right systolic function (tricuspid annulus movement < 10 mm and a prolonged RV pre-ejection period/pulmonary ejection time ratio) and six patients showed additional severe depression of parameters of diastolic function (pseudo-normal or restrictive RV filling pattems). Those patients with RV hypertrophy and severely compromised systolic and diastolic function had the highest LV masses (92 +/- 11.7 g/m(2.7)). CONCLUSION: RV involvement is common in Fabry disease and ultimately progresses to severe systolic and diastolic RV dysfunction. These findings might explain why patients with preserved LV function can develop clinical features such as reduced exercise capacity, organomegaly and lymphoedema. |
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Authors:
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C Kampmann; F A Baehner; C Whybra; M Bajbouj; K Baron; M Knuf; C M Wiethoff; H Trübel; M Beck |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Acta paediatrica (Oslo, Norway : 1992). Supplement Volume: 94 ISSN: 0803-5326 ISO Abbreviation: Acta Paediatr Suppl Publication Date: 2005 Mar |
Date Detail:
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Created Date: 2005-05-17 Completed Date: 2005-08-25 Revised Date: 2008-05-12 |
Medline Journal Info:
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Nlm Unique ID: 9315043 Medline TA: Acta Paediatr Suppl Country: Norway |
Other Details:
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Languages: eng Pagination: 15-8; discussion 9-10 Citation Subset: IM |
Affiliation:
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Department of Cardiology, University Children's Hospital, Mainz, Germany. christoph.kampmann@uni-mainz.de |
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Blood Pressure / physiology Body Mass Index Cardiomyopathies / complications, diagnosis, physiopathology Diagnosis, Differential Electrocardiography Fabry Disease / complications*, physiopathology* Female Heart Failure / complications, diagnosis, physiopathology Humans Hypertrophy, Left Ventricular / complications, pathology, physiopathology Hypertrophy, Right Ventricular / complications, pathology, physiopathology Male Ventricular Dysfunction, Right / complications*, diagnosis, physiopathology* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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