Quantification of coronary artery calcification in patients with FH using EBCT

Calcification of the coronary vessel wall is regarded as a marker of advanced coronary atherosclerosis.

To test whether patients with heterozygous familial hypercholesterolemia (FH) exhibit excessive calcification of the coronary vessel wall, we quantified coronary artery calcium in LDL-apheresis treated FH-patients with known severe coronary artery disease (CAD) (n = 10), in patients with moderate hypercholesterolemia and known severe CAD (n = 10), and in asymptomatic controls (n = 10) using electronic beam CT. The total coronary calcium score (Agatston-Score), the number of calcified lesions and the calcified plaque volume were evaluated for this study.

CAD-patients with FH, although on average 10 years younger, had a significantly higher total coronary calcium score (702/2018/2890), number of lesions (34/43/49) and calcified plaque volume (700/1818/2313) compared to patients with CAD only (480/641/1362, 10/16.5/22, 480/588/1209, respectively) and controls (10/47/137, 2/4/10, 15/50/144, respectively). Furthermore, we observed a significant correlation (r = 0.93; P < 0.01) between LDL-cholesterol levels (pretreatment levels of the CAD-FH group) and the total coronary calcium score in all three groups. Our results demonstrate that coronary artery calcification is more extensive in CAD-patients with FH than in CAD-patients with moderate hypercholesterolemia. In addition, we provide evidence that the amount of calcium in the coronary vessel wall in FH patients result from a long lasting history of elevated LDL-Cholesterol levels.

These findings emphasize the significance of LDL-cholesterol as a risk factor for atherosclerosis and underline the importance of early diagnosis of CAD and early cholesterol lowering therapy in FH patients.