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50 | Chapter 4 To evaluate which variables were associated with AoVC we used a univariable ordinal logistic regression model. Subsequently, we divided the AoVC-score (Agatston units) into three groups based on equal distribution of patients in whom AoVC was present: 1) AoVC-score = 0 (N = 190), 2) AoVC-score of > 0 – 37 (N = 43), and 3) AoVC-score > 37 (N = 43). Variables associated with AoVC where analyzed in the entire cohort with a multivariable ordinal logistic regressionmodel to identify a set of predictors of AoVC.We have chosen an ordinal regression model over a linear regression model to investigate a dose response relationship between the highly skewed AoVC variable and other variables. Correcting skewness by logistically transforming would have been possible as well, but we did not like to change the data into an artificial score in 190 persons whose Agatston score was 0. In addition, the association between the presence of CAC and AoVC was examined for he-FH patients and controls. The differences in distributions of AoVC in the presence and absence of CAC were analyzed with a Pearson Chi-Square test, and differences between the AoVC-score in the presence and absence of CAC with the Mann-Whitney-U test. Finally, we compared the influence of LDLR-negative, and LDLR-defective mutational he-FH on AoVC. Data were analyzed using SPSS (version 22, SPSS, Chicago, USA). Results Baseline characteristics of he-FH patients and controls Age, systolic blood pressure, and the prevalence of hypertension and diabetes were higher in control patients, than in the he-FH group (table 1). He-FH patients had higher untreated maxLDL levels, more frequently used statins, and more often reported a positive family history for CAD compared to the controls. Gender, body mass index and treated cholesterol levels were similar in both groups. AoVC in he-FH patients and controls AoVC was compared between he-FH patients and controls (table 2). AoVC was more prevalent in he-FH patients (41%, N = 59) than in controls (21%, N = 27, P < 0.001), irrespective of the age category. Limiting the analysis to patients with AoVC present, the AoVC-score (median, IQR) was higher in he-FH patients than in controls: 51 (9 - 117) and 21 (3 - 49), respectively (P = 0.007).

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