The association of epicardial fat thickness and disease severity among Fabry patients
The association of epicardial fat thickness and disease severity among Fabry patients
Aim: Patients with Fabry disease frequently die due to cardiovascular causes. There are numerous findings of cardiovascularinvolvement in patients with Fabry disease. Epicardial fat thickness is a surrogate for various cardiovascular diseases. In this study,we evaluated epicardial fat thickness and parameters of diastolic dysfunction among Fabry patients as a possible indicator ofdisease severity.Materials and Methods: We enrolled 14 patients with Fabry disease who receive enzyme replacement therapy. Detailedechocardiographic examination was performed including diastolic functions and measurement of epicardial fat thickness. Diseaseseverity was assessed using DS3 scoring system. Based on DS3 scores, patients are grouped into mild (
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- 1. Germain DP. Fabry disease. Orphanet J Rare Dis 2010;5:30
- 2. Baig S, Edward NC, Kotecha D, Liu B, Nordin S, Kozor R,
et al. Ventricular arrhythmia and sudden cardiac death in
Fabry disease: a systematic review of risk factors in clinical
practice. Europace 2017 doi: 10.1093/europace/eux261.
- 3. Iacobellis G. Local and systemic effects of the
multifaceted epicardial adipose tissue depot. Nat Rev
Endocrinol2015;11:363-71
- 4. Rozenfeld P, Feriozzi S. Contribution of inflammatory
pathways to Fabry disease pathogenesis. Mol Genet
Metab. 2017;122:19-27
- 5. Iacobellis G, Willens HJ. Echocardiographic epicardial
fat: a review of research and clinical applications. J Am
SocEchocardiogr 2009;22:1311-9
- 6. Nagueh SF, Smiseth OA, Appleton CP, Byrd BF 3rd, Dokainish
H, Edvardsen T, et al. Recommendations for the evaluation
of left ventricular diastolic function by echocardiography:
an update from American society of echocardiography and
the European association of cardiovascular imaging. J Am
Soc Echocardiogr 2016;29:277-314
- 7. Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A,
Ernande L, et al. Recommendations for cardiac chamber
quantification by echocardiography in adults: an update
from the American society of echocardiography and the
European association of cardiovascular imaging. J Am Soc
Echocardiogr 2015;28:1-39.
- 8. Giannini EH, Mehta AB, Hilz MJ, Beck M, Bichet DG, Brady
RO, et al. A validated disease severity scoring system for
Fabry disease. Mol Genet Metab 2010;99:283-90
- 9. Levey AS, Greene T, Kusek JW, Beck GL; MDRD Study Group.
A simplified equation to predict glomerular filtration rate
from serum creatinine. J Am Soc Nephrol 2000 Sep; 11:155A
- 10. Iacobellis G, Assael F, Ribaudo MC, et al. Epicardial fat from
echocardiography: a new method for visceral adipose tissue
prediction. Obes Res 2003;11:304-10.
- 11. Iacobellis G, Willens HJ, Barbaro G, et al. Threshold values
for high-risk echocardiographic epicardial fat thickness.
Obesity (Silver Spring) 2008;16:887-92.
- 12. Constricte A, Petramala L, Scoccia G, et al. Epicardial fat
thickness in patients with autosomal dominant polycystic
kidney disease. Cardiorenal Med 2018;8:199-207.
- 13. Iacobeliis G, Barbarini G, Letizia C, et al. Epicardial fat
thickness and nonalcoholic fatty liver disease in obese
subjects. Obesity (Silver Spring) 2014;22:332-6.
- 14. Gürdal A, Keskin K, Orken DN, et al. Evaluation of epicardial
fat thickness in young patients with embolic stroke of
undetermined source. Neurologist 2018;23:113-117.
- 15. Ahn SG, Lim HS, Joe DY, et al. Relationship of epicardial
adipose tissue by echocardiography to coronary artery
disease. Heart 2008;94:e7.
- 16. Eroglu S, Sade LE, Yildirir A, et al. Epicardial adipose tissue
thickness by echocardiography is a marker for the presence
and severity of coronary artery disease. Nutr Metab
Cardiovasc Dis 2009;19:211-7.
- 17. Park JS, Lee YH, Seo KW, et al. Echocardiographic epicardial
fat thickness is a predictor for target vessel revascularization
in patients with ST-elevation myocardial infarction. Lipids
Health Dis 2016;15:194.
- 18. Waldek S, Patel MR, Banikazemi M, et al. Life expectancy and
cause of death in males and females with Fabry disease:
findings from the Fabry Registry. Genet Med 2009;11:790-6.
- 19. Patel MR, Cecchi F, Cizmarik M, et al. Cardiovascular events
in patients with fabry disease natural history data from the
fabry registry. J Am Coll Cardiol 2011;57:1093-9.
- 20. Dabbah S, Komarov H, Marmor A, et al. Epicardial fat, rather
than pericardial fat, is independently associated with
diastolic filling in subjects without apparent heart disease.
Nutr Metab Cardiovasc Dis 2014;24:877-82.
- 21. Hachiya K, Fukuta H, Wakami K, et al. Relation of epicardial
fat to central aortic pressure and left ventricular diastolic
function in patients with known or suspected coronary
artery disease. Int J Cardiovasc Imaging 2014;30:1393-8.