Absence of Coronary Artery Calcification in Middle-Aged Familial Hypercholesterolemia Patients Without Atherosclerotic Cardiovascular Disease

Coronary lesion complexity in patients with heterozygous familial hypercholesterolemia hospitalized for acute myocardial infarction: data from the RICO survey

“…The percentage of optically healthy coronary arteries was much less frequent in FH patients than in non-FH patients, 3% vs 10% (P = 0.029), respectively, and 4 FH patients had coronary arteries without stenosis. Compared to the non-FH group, patients in the FH group had a higher initial SYNTAX score (11 [6-20] vs 8 [3][4][5][6][7][8][9][10][11][12][13][14][15], P = 0.005) and more frequent multivessel disease (56% versus 40%, P = 0.01) (Fig. 2).…”

“…Furthermore, FH patients have an unfavorable prognosis after MI, with a risk of recurrence of cardiovascular or coronary events that is 2 to 3 times higher than the average [9,10]. However, there are wide variations in the extent of CAD and in the level of coronary calcifications between individuals with genetically determined FH, suggesting the need for a better understanding of its specificities [4,11].…”

Role of coronary artery calcium score in the primary prevention of cardiovascular disease

“…The findings from these and other studies129 highlight the multifactorial nature of atherosclerosis, which is not only heterogenous among individuals with severe hypercholesterolemia but is also the main driver of future clinical risk. While we do not advocate cessation of, or refraining from statins in those with severely elevated levels of LDL-C (indeed, a large proportion of participants in the aforementioned studies were treated with statins, including many of those with CAC=0), CAC can provide valuable insight into risk stratification strategies for a more cost effective allocation of novel, costly lipid lowering treatments 130131132.…”

Correlates of Coronary Artery Calcification Prevalence and Severity in Patients With Heterozygous Familial Hypercholesterolemia

“…These numbers are consistent with data from a meta-analysis of 9 studies in which the overall prevalence of CAC score > 0 AU was estimated to be 55% (95% CI: 45%-66%) among patients with HeFH free of CHD. 39 Considering that CAC is one of the most potent predictors of CHD, 8,19 the fact that about half of individuals with HeFH present no CAC demonstrates the heterogeneity in CHD risk profiles in this population, 5,[40][41][42][43] and underscores the importance of documenting the determinants of atherosclerosis in this population. In that regard, our results support currently recommended management approaches including effective cascade screening strategies, early treatment onset, and lifestyle management education comprising counseling on diet and smoking cessation, independent of the prevalence and severity of CAC.…”