Article Text

PDF
Coronary microvascular dysfunction in male patients with Anderson-Fabry disease and the effect of treatment with α galactosidase A
  1. P M Elliott1,
  2. H Kindler2,
  3. J S Shah1,
  4. B Sachdev1,
  5. O E Rimoldi2,
  6. R Thaman1,
  7. M T Tome1,
  8. W J McKenna1,
  9. P Lee3,
  10. P G Camici2
  1. 1The Heart Hospital, University College London, London, UK
  2. 2MRC Clinical Sciences Centre, Imperial College, London, UK
  3. 3The Charles Dent Metabolic Unit, The National Hospital for Neurology and Neurosurgery, London, UK
  1. Correspondence to:
    Dr Perry M Elliott
    The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, UK; perry.elliott{at}uclh.nhs.uk

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 and results: Mean (SD) myocardial blood flow (MBF) at rest and during hyperaemia (adenosine 140 μg/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.

  • AFD, Anderson-Fabry disease
  • CFR, coronary flow reserve
  • ERT, enzyme replacement therapy
  • LV, left ventricular
  • MBF, myocardial blood flow
  • PET, positron emission tomography
  • cardiomyopathy
  • coronary circulation
  • myocardial blood flow
  • myocardial ischaemia
  • cardiac imaging

Statistics from Altmetric.com

Footnotes

  • Published Online First 4 August 2005

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.