Document Detail

Cardiac performance during exercise in patients with Fabry's disease.
MedLine Citation:
PMID:  19021715     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Fatigability and dyspnoea on effort are present in many patients with Fabry's disease. We assessed the determinants of cardiac performance during exercise in patients with Fabry's disease and preserved left ventricular ejection fraction at rest. MATERIALS AND METHODS: Sixteen patients with Fabry's disease and 16 control subjects underwent radionuclide angiography at rest and during exercise, tissue Doppler echocardiography and magnetic resonance imaging at rest. RESULTS: The exercise-induced change in stroke volume was +25 +/- 14% in controls and +5.8 +/- 19% in patients with Fabry's disease (P < 0.001). In 10 patients (group 1), the stroke volume increased (+19 +/- 10%), and in 6 patients (group 2) it decreased (-16 +/- 9%) with exercise. Patients of group 2 were older, had worse renal function, higher left ventricular mass and impaired diastolic function compared to group 1. The abnormal stroke volume response to exercise in group 2 was associated with a decrease in end-diastolic volume (P < 0.001) and a lack of reduction of end-systolic volume (P < 0.01) compared with both controls and group 1. The ratio of peak early-diastolic velocity from mitral filling to peak early-diastolic mitral annulus velocity was the only independent predictor of exercise-induced change in stroke volume (B -0.44; SE 0.119; beta-0.70; P < 0.005). CONCLUSIONS: The majority of patients with Fabry's disease were able to augment stroke volume during exercise by increasing end-diastolic volume, whereas patients with more advanced cardiac involvement may experience the inability to increase cardiac output by the Frank Starling mechanism.
L Spinelli; E Nicolai; W Acampa; M Imbriaco; A Pisani; M A E Rao; F Scopacasa; B Cianciaruso; N De Luca; A Cuocolo
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  European journal of clinical investigation     Volume:  38     ISSN:  1365-2362     ISO Abbreviation:  Eur. J. Clin. Invest.     Publication Date:  2008 Dec 
Date Detail:
Created Date:  2008-11-21     Completed Date:  2009-06-16     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0245331     Medline TA:  Eur J Clin Invest     Country:  England    
Other Details:
Languages:  eng     Pagination:  910-7     Citation Subset:  IM    
Department of Internal Medicine, Cardiovascular and Immunological Sciences, University Federico II, Naples, Italy.
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MeSH Terms
Aged, 80 and over
Cardiac Output, Low / physiopathology*
Case-Control Studies
Exercise Test*
Exercise Tolerance / physiology*
Fabry Disease / genetics,  physiopathology*
Middle Aged
Prospective Studies
Stroke Volume / physiology*
Ventricular Function, Left / physiology*

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