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Cardiac performance during exercise in patients with Fabry's disease
L Spinelli1, E Nicolai, W Acampa
1Department of Internal Medicine, Cardiovascular and Immunological Sciences, University Federico II, Naples, Italy.
European Journal of Clinical Investigation
|November 22, 2008
Summary
Patients with Fabry disease show impaired stroke volume response during exercise. Diastolic dysfunction predicts reduced cardiac output, impacting exercise capacity in advanced cardiac involvement.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Fabry disease often presents with exercise intolerance, including fatigability and dyspnoea.
- This study investigates cardiac performance determinants during exercise in Fabry disease patients with preserved resting ejection fraction.
Purpose of the Study:
- To assess the factors influencing cardiac performance during exercise in Fabry disease patients.
- To identify predictors of abnormal stroke volume response to exercise in this population.
Main Methods:
- Sixteen Fabry disease patients and 16 controls underwent radionuclide angiography (rest and exercise), echocardiography, and MRI.
- Tissue Doppler echocardiography was used to assess diastolic function.
Main Results:
- Fabry patients exhibited a significantly blunted increase in stroke volume during exercise compared to controls.
- A subset of patients (group 2) showed a decrease in stroke volume with exercise, linked to older age, worse renal function, higher LV mass, and impaired diastolic function.
- Impaired diastolic function, specifically the ratio of early diastolic mitral inflow to mitral annular velocity, was the sole independent predictor of abnormal exercise-induced stroke volume changes.
Conclusions:
- Most Fabry patients can increase stroke volume via end-diastolic volume augmentation during exercise.
- Patients with advanced cardiac involvement may lose the ability to increase cardiac output through the Frank-Starling mechanism due to impaired diastolic function.
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