Aortic Calcification Progression in Heterozygote Familial Hypercholesterolemia

Manal Al Kindi, Alexandre M. Bélanger, Karl Sayegh, Soumia Senouci, Sumayah Aljenedil, Lojan Sivakumaran, Isabelle Ruel, Khalid Al Rasadi, Khalid Al Waili, Zuhier Awan, David Valenti, Jacques Genest

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Abstract

Background Patients with homozygous and heterozygous familial hypercholesterolemia (HeFH) develop severe aortic calcifications in an age- and gene dosage-dependent manner. The purpose of this study was to determine the rate of progression of aortic calcification in patients with HeFH. Methods We performed thoracoabdominal computed tomography scans and quantified aortic calcium (AoCa) score in 16 HeFH patients, all with the null low-density lipoprotein (LDL) receptor DEL15Kb mutation. Patients (12 men, 4 women) were rescanned an average of 8.2 ± 0.8 years after the first scan. Results Mean LDL cholesterol (LDL-C) during treatment was 2.53 mmol/L; all patients were receiving high-dose statin/ezetimibe; 5 of 16 were receiving evolocumab. Baseline LDL-C was 7.6 ± 1.3 mmol/L. Aortic calcifications increased in all patients in an exponential fashion with respect to age. Age was the strongest correlate of AoCa score. Cholesterol, LDL-C, or age × cholesterol did not correlate with AoCa score or its progression. Control patients (n = 31; 8 male, 23 female; mean age 61 ± 11 years) who underwent virtual colonoscopy were rescanned over the same period and showed an abdominal AoCa score of 1472 ± 2489 compared with 7916 ± 7060 Agatston U (P < 0.001) in patients with HeFH during treatment (mean age, 60 ± 14 years). The rate of progression was 159 vs 312 Agatston U/y in control participants vs those with HeFH. Conclusions HeFH patients exhibit accelerated aortic calcification that increases exponentially with age. LDL-C at baseline or during treatment seems to have little effect on the rate of progression of AoCa score. Strategies to prevent aortic calcifications with statins have not met with clinical success and novel approaches are required; statins might also contribute to the process of arterial calcification.

Original languageEnglish
Pages (from-to)658-665
Number of pages8
JournalCanadian Journal of Cardiology
Volume33
Issue number5
DOIs
Publication statusPublished - May 1 2017

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Hyperlipoproteinemia Type II
Heterozygote
Hydroxymethylglutaryl-CoA Reductase Inhibitors
Calcium
LDL Lipoproteins
LDL Cholesterol
Computed Tomographic Colonography
Gene Dosage
LDL Receptors
Therapeutics
Cholesterol
Tomography
Mutation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Al Kindi, M., Bélanger, A. M., Sayegh, K., Senouci, S., Aljenedil, S., Sivakumaran, L., ... Genest, J. (2017). Aortic Calcification Progression in Heterozygote Familial Hypercholesterolemia. Canadian Journal of Cardiology, 33(5), 658-665. https://doi.org/10.1016/j.cjca.2017.02.001

Aortic Calcification Progression in Heterozygote Familial Hypercholesterolemia. / Al Kindi, Manal; Bélanger, Alexandre M.; Sayegh, Karl; Senouci, Soumia; Aljenedil, Sumayah; Sivakumaran, Lojan; Ruel, Isabelle; Al Rasadi, Khalid; Al Waili, Khalid; Awan, Zuhier; Valenti, David; Genest, Jacques.

In: Canadian Journal of Cardiology, Vol. 33, No. 5, 01.05.2017, p. 658-665.

Research output: Contribution to journalArticle

Al Kindi, M, Bélanger, AM, Sayegh, K, Senouci, S, Aljenedil, S, Sivakumaran, L, Ruel, I, Al Rasadi, K, Al Waili, K, Awan, Z, Valenti, D & Genest, J 2017, 'Aortic Calcification Progression in Heterozygote Familial Hypercholesterolemia', Canadian Journal of Cardiology, vol. 33, no. 5, pp. 658-665. https://doi.org/10.1016/j.cjca.2017.02.001
Al Kindi M, Bélanger AM, Sayegh K, Senouci S, Aljenedil S, Sivakumaran L et al. Aortic Calcification Progression in Heterozygote Familial Hypercholesterolemia. Canadian Journal of Cardiology. 2017 May 1;33(5):658-665. https://doi.org/10.1016/j.cjca.2017.02.001
Al Kindi, Manal ; Bélanger, Alexandre M. ; Sayegh, Karl ; Senouci, Soumia ; Aljenedil, Sumayah ; Sivakumaran, Lojan ; Ruel, Isabelle ; Al Rasadi, Khalid ; Al Waili, Khalid ; Awan, Zuhier ; Valenti, David ; Genest, Jacques. / Aortic Calcification Progression in Heterozygote Familial Hypercholesterolemia. In: Canadian Journal of Cardiology. 2017 ; Vol. 33, No. 5. pp. 658-665.
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AB - Background Patients with homozygous and heterozygous familial hypercholesterolemia (HeFH) develop severe aortic calcifications in an age- and gene dosage-dependent manner. The purpose of this study was to determine the rate of progression of aortic calcification in patients with HeFH. Methods We performed thoracoabdominal computed tomography scans and quantified aortic calcium (AoCa) score in 16 HeFH patients, all with the null low-density lipoprotein (LDL) receptor DEL15Kb mutation. Patients (12 men, 4 women) were rescanned an average of 8.2 ± 0.8 years after the first scan. Results Mean LDL cholesterol (LDL-C) during treatment was 2.53 mmol/L; all patients were receiving high-dose statin/ezetimibe; 5 of 16 were receiving evolocumab. Baseline LDL-C was 7.6 ± 1.3 mmol/L. Aortic calcifications increased in all patients in an exponential fashion with respect to age. Age was the strongest correlate of AoCa score. Cholesterol, LDL-C, or age × cholesterol did not correlate with AoCa score or its progression. Control patients (n = 31; 8 male, 23 female; mean age 61 ± 11 years) who underwent virtual colonoscopy were rescanned over the same period and showed an abdominal AoCa score of 1472 ± 2489 compared with 7916 ± 7060 Agatston U (P < 0.001) in patients with HeFH during treatment (mean age, 60 ± 14 years). The rate of progression was 159 vs 312 Agatston U/y in control participants vs those with HeFH. Conclusions HeFH patients exhibit accelerated aortic calcification that increases exponentially with age. LDL-C at baseline or during treatment seems to have little effect on the rate of progression of AoCa score. Strategies to prevent aortic calcifications with statins have not met with clinical success and novel approaches are required; statins might also contribute to the process of arterial calcification.

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