Coronary artery bypass grafting for Fabry's disease: veins more suitable than arteries?

Chimenti, Cristina; Morgante, Emanuela; Critelli, G; Russo, Matteo Antonio; Frustaci, Andrea

doi:10.1016/j.humpath.2007.07.001

Cited by 13 publications

(11 citation statements)

References 8 publications

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“…[38][39][40] No atherosclerosis of significance was observed, confirming observations by others. 6,7,[41][42][43] Increased IMT was related some disease manifestations but lost significance when adjusted for age and sex. In general, an increase in IMT is associated with an increased risk of stroke.…”

Section: Discussionmentioning

confidence: 96%

Vascular Aspects of Fabry Disease in Relation to Clinical Manifestations and Elevations in Plasma Globotriaosylsphingosine

et al. 2012

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Abstract-Fabry disease is an X-linked hereditary lysosomal storage disorder attributed to a deficiency of α-galactosidaseA leading to increased plasma levels of globotriaosylsphingosine (lysoGb3 01).In women and atypical patients these vascular parameters were comparable with controls. Pulse wave velocity was not different; advanced glycation end products were only slightly increased in atypical patients. In classically affected women, a small increase in lysoGb3 was associated with an increase in IMT independent of age.In the classically affected men, all with increased IMT and high levels of plasma lysoGb3, lysoGb3 levels did not add to a higher IMT, suggestive of a ceiling effect. For FMD, elevated lysoGb3 levels (>7 nmol/L) contributed to a 2.9% lower FMD independent of age and sex (P=0.02). Increased carotid IMT and decreased brachial FMD occur in classic Fabry disease, which is associated with plasma lysoGb3 level independent of age and sex. These observations still exist despite enzyme replacement therapy. (Hypertension. 2012;60:998-1005.)

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Section: Discussionmentioning

confidence: 96%

Vascular Aspects of Fabry Disease in Relation to Clinical Manifestations and Elevations in Plasma Globotriaosylsphingosine

et al. 2012

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“…5,6 Involvement of large muscular arteries (coronary, renal, and intrarenal) in Fabry disease patients has been characterized by accumulation of GL-3 storage deposits in endothelium and smooth muscle cells of the media. [11][12][13][14][15] Progression of vasculopathy results in formation of a thickened fibrocellular intima containing fibroblasts with storage deposits and fibrosis and calcification of the media. [11][12][13][14][15] This form of vasculopathy has features of a type of fibromuscular dysplasia or a type of arteriosclerosis as literally defined rather than typical atherosclerosis of the elastic and muscular arteries.…”

Section: Article See P 2561mentioning

confidence: 99%

“…[11][12][13][14][15] Progression of vasculopathy results in formation of a thickened fibrocellular intima containing fibroblasts with storage deposits and fibrosis and calcification of the media. [11][12][13][14][15] This form of vasculopathy has features of a type of fibromuscular dysplasia or a type of arteriosclerosis as literally defined rather than typical atherosclerosis of the elastic and muscular arteries. 5,6 Although GL-3 uptake is via the LDL receptor, lipid accumulation in evolving atherosclerotic lesions involves local oxidation of LDL and poorly regulated cellular uptake of the oxidized LDL via scavenger pathway receptors, not the LDL receptor.…”

Section: Article See P 2561mentioning

confidence: 99%

“…An interesting case report described occlusion of a left internal mammary artery graft while a saphenous vein bypass graft remained patent in a Fabry disease patient with coronary artery disease. 15 Factors promoting occlusion of the left internal mammary artery may have been the arterial pressure promoting GL-3 deposition as well as endothelial dysfunction favoring thrombosis. 15 The current report of Thurberg et al does not directly address the status of the pathophysiology or anatomy of the epicardial coronary arteries in this group of 58 patients with Fabry disease.…”

Section: Article See P 2561mentioning

confidence: 99%

“…15 Factors promoting occlusion of the left internal mammary artery may have been the arterial pressure promoting GL-3 deposition as well as endothelial dysfunction favoring thrombosis. 15 The current report of Thurberg et al does not directly address the status of the pathophysiology or anatomy of the epicardial coronary arteries in this group of 58 patients with Fabry disease. 4 Also, the report is silent on the presence or absence of changes in myocardial vessels larger than capillaries, such as arterioles and small arteries, which may have been randomly sampled in the small EMBs.…”

Section: Article See P 2561mentioning

confidence: 99%

See 2 more Smart Citations

Evaluation of Recombinant α-Galactosidase A Therapy for Amelioration of the Cardiovascular Manifestations of Fabry Disease

Buja

2009

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Anderson-Fabry disease in heart failure

Akhtar

Elliott

2018

Biophys Rev

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Anderson-Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the GLA gene that result in deficiency of the enzyme alpha-galactosidase A. The worldwide incidence of Fabry’s disease is reported to be in the range of 1 in 40,000–117,000, although this value may be a significant underestimate given under recognition of symptoms and delayed or missed diagnosis. Deficiency in alpha-galactosidase A causes an accumulation of neutral glycosphingolipids such as globotriaosylceramide (Gb3) in lysosomes within various tissues including the vascular endothelium, kidneys, heart, eyes, skin and nervous system. Gb3 accumulation induces pathology via the release of pro-inflammatory cytokines, growth-promoting factors and by oxidative stress, resulting in myocardial extracellular matrix remodelling, left ventricular hypertrophy (LVH), vascular dysfunction and interstitial fibrosis. Cardiac involvement manifesting as ventricular hypertrophy, systolic and diastolic dysfunction, valvular abnormalities and conduction tissue disease is common in AFD and is associated with considerable cardiovascular morbidity and mortality from heart failure, sudden cardiac death and stroke-related death.

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Coronary artery bypass grafting for Fabry's disease: veins more suitable than arteries?

Cited by 13 publications

References 8 publications

Vascular Aspects of Fabry Disease in Relation to Clinical Manifestations and Elevations in Plasma Globotriaosylsphingosine

Vascular Aspects of Fabry Disease in Relation to Clinical Manifestations and Elevations in Plasma Globotriaosylsphingosine

Evaluation of Recombinant α-Galactosidase A Therapy for Amelioration of the Cardiovascular Manifestations of Fabry Disease

Anderson-Fabry disease in heart failure

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