Aortic Root Calcification Score as an Independent Factor for Predicting Major Adverse Cardiac Events in Familial Hypercholesterolemia.

Aim: The aims of this study were: 1) to determine whether the accumulation of aortic root calcification (ARC) assessed using coronary computed tomography angiography (CCTA) can predict future cardiovascular events, and 2) to estimate the onset and progression of ARC in patients with familial hypercholesterolemia (FH).

Methods: One hundred thirteen consecutive Japanese patients with heterozygous FH (male=54, mean age=52.1±15.6 years, mean LDL-C=299.0±94.6 mg/dL), without known coronary artery disease, who underwent 64-detector row CCTA were retrospectively evaluated. ARC was defined as the presence of calcium at the aortic root. The extent of ARC was expressed in Agatston units as the ARC-score. Major adverse cardiac events (MACE) were defined as either cardiac death, ST elevated myocardial infarction (STEMI), non-ST elevated myocardial infarction (NSTEMI), unstable angina pectoris (UAP), planned percutaneous coronary intervention (PCI), coronary artery bypass grafting (CABG), or stroke. The periods to MACE were estimated using multivariate logistic regression analysis.

Results: During the follow-up period (median 1635 days), 19 instances of MACE occurred. Multivariate logistic regression analysis revealed that ARC was a significant independent predictor of MACE (OR=1.48; 95% CI 1.11-1.87, p＜0.001, respectively). The regression equations were Y=0.09X- 1.59 (R=0.34, p＜0.001) in males and Y=0.08X-1.60 (R=0.13, p＜0. 05) in females.

Conclusions: ARC was significantly associated with future MACE in Japanese patients with heterozygous FH. ARC may start to develop, on average, at 17.4 and 19.7 years of age in males and females, respectively, with heterozygous FH.