Presented by: Lilton R.C. Martinez MD, Marcio H. Miname MD, Luiz A. Bortolotto MD, Ana P.M. Chacra MD, Carlos E. Rochitte MD, Andrei C. Sposito MD, Raul D. Santos MD,PhD.

1. No correlation and low agreement of imaging and inflammatory atherosclerosis’ markers in familial hypercholesterolemia Lilton R.C. Martinez MD, Marcio H. Miname MD, Luiz A. Bortolotto MD, Ana P.M. Chacra MD, Carlos E. Rochitte MD, Andrei C. Sposito MD, Raul D. Santos MD,PhD. Lipid Clinic Heart Institute InCor-University of Sao Paulo Medical School Hospital Sao Paulo, Brazil Martinez LRC et al . Atherosclerosis (2008), doi:10.1016/j.atherosclerosis.2007.12.014

14. Table 3: CAC, IMT and PWV in FH patients and NL subjects. Martinez LRC et al . Atherosclerosis (2008), doi:10.1016/j.atherosclerosis.2007.12.014 0.026 0.02 0 (0-54) 4.5 ± 13 0 (0-798) 46 ± 140 CCS Agatston units median (ranges) mean ± SD 0.87 6623 ± 796 6596 ± 772 Carotid diameter (μm) 0.99 460 ± 178 461 ± 194 Carotid distension (μm) 0.027 593 ± 111 653 ± 160 Carotid IMT (μm) 0.007 8.5 ± 1 9.2 ± 1.5 PWV (m/s) 0.041 1 (4%) 21 (23%) CCS ≥ 75 th % 0.024 3 (12%) 30 (34%) CAC prevalence n (%) P NL (n=31) FH (n=89)

15. Table 4: Univariate determinants of CAC as continuous and dichotomous variable, IMT and PWV in FH patients 0.08 - - - - - Male gender CAC dichotomous p= p = Log(CCS+1) r = p = PWV r = p = IMT r = Determinants 0.001 0.002 0.33 - - 0.0001 0.55 Carotid diameter (μm) 0.014 0.007 0.29 - - - - PWV (m/s) - 0.02 0.26 0.0008 0.35 - - IMT (μm) - - - 0.003 0.32 0.02 0.26 Log (CCS+1) - 0.04 0.23 - - Apolipoprotein A-I (g/l) 0.0036 0.008 0.28 0.02 0.26 - - Lp(a) (mg/dl) - - - - 0.05 0.22 Apolipoprotein B (g/l) - - - - 0.01 0.28 CRP (mg/l) - - - 0.01 0.26 0.02 0.26 Glucose (mg/dl) 0.024 0.02 0.25 - - 0.03 0.24 TG / HDL- cholesterol 0.02 0.01 0.27 0.03 0.24 0.03 0.23 TG (mg/dl) - 0.04 0.22 - - - - LDL-cholesterol /HDL- cholesterol 0.044 0.02 0.25 - - - - LDL-cholesterol (mg/dl) 0.02 0.25 - - - - Total cholesterol (mg/dl) <0.001 0.0001 0.51 0.0001 0.52 0.003 0.32 LYS <0.001 0.0001 0.48 0.0002 0.39 0.006 0.29 Civiera (high risk) <0.001 0.0001 0.54 0.0001 0.44 0.0001 0.40 FRS - - - - 0.005 0.31 PP (mmHg) - - 0.01 0.27 0.009 0.28 DBP. (mmHg) - - 0.02 0.26 0.0003 0.39 SBP (mmHg) 0.04 - - - - - - Xanthoma 0.001 - - - - - - Metabolic Syndrome <0.001 0.0001 0.43 0.0001 0.62 0.002 0.33 Age (years)

20. Table 5: Agreement degree of imaging and inflammatory markers severity in FH subjects Martinez LRC et al . Atherosclerosis (2008), doi:10.1016/j.atherosclerosis.2007.12.014 With exception of moderate agreement between IMT and PWV severity (kappa = 0.517) all other markers of severity presented only a slight agreement (kappa < 0.1). 0.031 CRP > 3 (mg/l) PWV > 12 (m/s) 0.064 CRP > 3 (mg/l) IMT > 900 (μm) 0.517 PWV > 12 (m/s) IMT > 900 (μm) 0.16 CRP > 3 (mg/l) CCS > 75 th % 0.004 PWV > 12 (m/s) CCS > 75 th % 0.109 IMT > 900 (μm) CCS > 75 th % kappa