ARTERIAL STIFFNESS IN FAMILIAL HYPERCHOLESTEROLEMIA: ARE THERE ANY RISKS IN CHILDHOOD
Authors:   Kazan State Medical University, Kazan, Republic of Tatarstan, Russia, Galimova L.F. ,   Children's Republican Clinical Hospital with the Ministry of Healthcare of the Republic of Tatarstan, Kazan, Republic of Tatarstan, Russia, Sadykova D.I. ,   Kazan State Medical University, Kazan, Republic of Tatarstan, Russia, Slastnikova E.S. ,   Kazan State Medical University, Kazan, Republic of Tatarstan, Russia,   Children's Republican Clinical Hospital with the Ministry of Healthcare of the Republic of Tatarstan, Kazan, Republic of Tatarstan, Russia, Marapov D.I. ,   Kazan State Medical Academy, Branch of the Russian Medical Academy for Continuous Professional Education, Kazan, Republic of Tatarstan, Russia, Guseva N.E. ,   Kazan State Medical University, Kazan, Republic of Tatarstan, Russia,   Children's Republican Clinical Hospital with the Ministry of Healthcare of the Republic of Tatarstan, Kazan, Republic of Tatarstan, Russia, Haliullina Ch.D. ,   Kazan State Medical University, Kazan, Republic of Tatarstan, Russia
Published: March 2022

The most common monogenic hereditary disease is familial hypercholesterolemia (FH), in which an abnormal increase in blood cholesterol levels has been observed since childhood and can lead to the development of premature atherosclerosis. Indicators of arterial stiffness can be used as independent markers of structural and morphological changes in the vascular wall. Objective of the study: to study the features of arterial stiffness in children with heterozygous FH. Materials and methods of research: an open prospective cohort single-center study was conducted from January 2017 to August 2021. It involved 87 children aged 5 to 17 years [median 11 years (7.0–15.0 years)], of which 44 children (21 boys and 23 girls) were diagnosed with FH, heterozygous form (set in accordance with Simon Broome British criteria), who did not undergo lipid-lowering therapy (the main group), and 43 healthy children (22 boys and 21 girls) included in the control group. Group differences in age and gender were not statistically significant (p=0.99 and p=0.749, respectively). All children underwent daily monitoring of blood pressure equipped with the option of measuring arterial stiffness Vasotens («Peter Telegin» LLC, Nizhny Novogorod). The pulse wave velocity in the aorta (PWVao), augmentation index (AIx), and the maximum rate of pressure rise (dp/dt) were evaluated. Results: children with FH showed a statistically significant increase in aortic pulse wave velocity (PWVao) compared to healthy peers: minimal [3.85 (3.3–5.25) m/s versus 3.3 (2.8–3.9) m/s, p=0.009], medium [6.8 (5.8–8.6) m/s versus 4.3 (3.7–5.1) m/s, p<0.001] and maximum per day [8.25 (7.2–11.2) m/s versus 6.3 (5.15–7.25) m/s, p<0.001]. In the FH group, compared with the control group, a statistically significant increase in the minimum [–69.61±8.08 (–72.07–(–67.16))% and –73.05±7.32 (–75.3–(–70.8))%, respectively, р=0.041] and the daily average augmentation index [–44.5 (–39.0–(–34.0))% and –50.0 (–59.5–(–44.0))% respectively, р<0.001], medium [628.0 (582.0–730.0) mmHg/s and 569.0 (387.0–644.0) mmHg/s, respectively, p=0.001] and the maximum per day maximum rate of pressure rise [1109.5 (1030.8–1475.0) mmHg/s and 965.0 (667.5–1002.5) mmHg/s, respectively, p<0.001]. Correlation analysis in the FH group showed statistically significant direct correlation between low density lipoprotein (LDL) content in the blood serum and maximum PWVao (r=0.485, p<0.001) as well as between the LDL content in the blood serum and the average PWVao (r=0.492, p<0.001). Conclusion: the results show, along with the increase in arterial stiffness in patients with FH, their relationship to the level of LDL, which allows to consider them as a possible additional method of determining cardiovascular risk in children with FH and assessing disease progression.