Coronary microvascular dysfunction in male patients with Anderson-Fabry disease and the effect of treatment with alpha galactosidase A.
Heart
; 92(3): 357-60, 2006 Mar.
Article
en En
| MEDLINE
| ID: mdl-16085718
ABSTRACT
OBJECTIVE:
To measure coronary flow reserve (CFR), an index of microvascular function, in Anderson-Fabry disease (AFD) at baseline and after enzyme replacement therapy (ERT). METHODS ANDRESULTS:
Mean (SD) myocardial blood flow (MBF) at rest and during hyperaemia (adenosine 140 microg/kg/min) was measured in 10 male, non-smoking patients (53.8 (10.9) years, cholesterol 5.5 (1.3) mmol/l) and in 24 age matched male, non-smoking controls (52.0 (7.6) years, cholesterol 4.5 (0.6) mmol/l) by positron emission tomography (PET). Resting and hyperaemic MBF and CFR (hyperaemic/resting MBF) were reduced in patients compared with controls (0.99 (0.17) v 1.17 (0.25) ml/g/min, p < 0.05; 1.37 (0.32) v 3.44 (0.78) ml/g/min, p < 0.0001; and 1.41 (0.39) v 3.03 (0.85), p < 0.0001, respectively). This coronary microvascular dysfunction was independent of cholesterol concentrations. PET was repeated in five patients after 10.1 (2.3) months of ERT; resting and hyperaemic MBF and CFR were unchanged after ERT (0.99 (0.16) v 0.99 (0.16) ml/g/min; 1.56 (0.29) v 1.71 (0.3) ml/g/min; and 1.6 (0.37) v 1.74 (0.28), respectively; all not significant).CONCLUSIONS:
The results of the present study show that patients with AFD have very abnormal coronary microvascular function. These preliminary data suggest that ERT has no effect on coronary microvascular dysfunction. Further work is necessary to determine whether treatment at an earlier stage in the course of the disease may improve coronary microvascular function in patients with AFD.
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Enfermedad de Fabry
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Alfa-Galactosidasa
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Circulación Coronaria
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Enfermedad Coronaria
Tipo de estudio:
Observational_studies
/
Risk_factors_studies
Límite:
Adult
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Aged
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Aged80
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Heart
Asunto de la revista:
CARDIOLOGIA
Año:
2006
Tipo del documento:
Article
País de afiliación:
Reino Unido