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Cardiac performance during exercise in patients with Fabry's disease
Author(s) -
Spinelli L.,
Nicolai E.,
Acampa W.,
Imbriaco M.,
Pisani A.,
Rao M. A. E.,
Scopacasa F.,
Cianciaruso B.,
De Luca N.,
Cuocolo A.
Publication year - 2008
Publication title -
european journal of clinical investigation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.164
H-Index - 107
eISSN - 1365-2362
pISSN - 0014-2972
DOI - 10.1111/j.1365-2362.2008.02053.x
Subject(s) - medicine , cardiology , ejection fraction , stroke volume , fabry disease , diastole , stroke (engine) , doppler echocardiography , end diastolic volume , cardiac output , end systolic volume , radionuclide angiography , hemodynamics , heart failure , disease , blood pressure , mechanical engineering , engineering
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; β–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.