Background and Aims: The aim of the present analysis of RENAISSANCE registry (Registry of patients with familial hypercholesterolemia (FH) and very high cardiovascular risk with lack of efficiency of lipid-lowering therapy) is the assessment of adherence and efficiency of the lipid-lowering therapy of patients with FH.

Цель. Целью исследования явилась оценка эффективности и приверженности гиполипидемической терапии, частоты развития сердечно<со< судистых осложнений в течение 3<летнего периода наблюдения в рамках регистра РЕНЕССАНС (Регистр пациентов с СГХС и пациентов очень высокого сЕрдечно<Сосудистого риска с недоСтАточной эффективНоСтью проводимой гиполипидемической терапии). Материал и методы. РЕНЕССАНС является открытым национальным наблюдательным исследованием и включает больных с семейной ги< перхолестеринемией (СГХС), а также пациентов очень высокого сердечно<сосудистого риска (ОВССР). Учитывали наличие факторов риска атеросклероза, анамнез сердечно<сосудистых заболеваний, гиполипидемическую терапию. В каждом центре выполняли определение концен< трации: общего холестерина (ОХС), триглицеридов (ТГ), холестерина липопротеидов высокой плотности (ХС ЛВП) в сыворотке крови. Содер< жание холестерина липопротеидов низкой плотности (ХС ЛНП) рассчитывали по формуле Фридвальда. Уровень липопротеида(а) измеряли методом иммуноферментного анализа в некоторых центрах. При оценке частоты конечной точки, включавшей фатальные и нефатальные сердечно<сосудистых осложнения (ССО), проводили анализ Каплана — Майера. Результаты. В регистр включено 1570 (средний возраст 54,0±14,6 лет) пациентов с СГХС и 121 (63,5±10,9 лет) больной с ОВССР. В группе СГХС динамическое наблюдение проведено у 594 пациентов (38%) в течение 23,6±14,6 месяцев, конечная точка зарегистрирована у 9% больных. Мужской пол (относительный риск 2,1; 95% доверительный интервал 1,13–3,66; p<0,01), гипертония (2,8; 1,4–5,2; p<0,01), ишеми< ческая болезнь сердца (6,8; 3,5–13,2; p<0,0001), отягощенный анамнез по сердечно<сосудистым заболеваниям (ССЗ) (2,1; 1,1–3,9; p<0,05) и концентрация липопротеида(а) ≥ 30 мг/дл (2,8; 1,1–7,7; p<0,05) явились предикторами развития ССО. В группе СГХС отмечено снижение уровня ОХС от исходного на 19%, ХС ЛНП на 25% (р<0,001 для обоих), целевых значений ХС ЛНП достигли 2% больных. В группе ОВССР динамическое наблюдение проведено у 72 (60%) пациентов в течение 19,7±5,8 месяцев. Ни один больной не достиг целевого уровня ХС ЛНП менее 1,4 ммоль/л. Заключение. Трехлетнее наблюдение за участниками регистра РЕНЕССАНС демонстрирует усиление приверженности гиполипидемической терапии. С увеличением риска развития сердечно<сосудистых осложнений при СГХС ассоциированы мужской пол, наличие гипертонии, ише< мической болезни сердца, отягощенного анамнеза по ССЗ и высокий уровень липопротеида(а). The aim of the study was to evaluate the effectiveness and adherence to hypolipidemic therapy, the frequency of cardiovascular events (CVE) during the 3!year follow!up in the RENAISSANCE registry (Registry of patients with familial hypercholesterolemia and very high cardiovascular risk with insufficient effect of hypolipidemic therapy). Methods. The RENAISSANCE registry is an open, national, observational study and includes patients with familial hypercholesterolemia (FH), as well as patients of very high cardiovascular risk (VHR). We took into consideration atherosclerosis risk factors and history of cardiovascular diseases (CVD), adherence to hypolipidemic therapy. Concentrations of total cholesterol (TC), triglycerides (TG), high density lipoprotein cholesterol (HDL!C) were measured in blood serum in all centers. Low density lipoprotein cholesterol (LDL!C) level was defined according to Friedewald formula. The concentration of lipoprotein(a) was measured by enzyme!linked immunosorbent assay in serum in some centers. Kaplan!Mayer analysis was performed to assess the frequency of fatal and nonfatal CVE. Results. The Registry consisted of 1570 (mean age 54.0±14.6 years) FH patients and 121 (63.5±10.9 years) VHR patients. Data of 594 patients (38%) who had follow!up visits were obtained in FH patients, follow!up duration 23.6±14.6 months, 54 (9%) patients experienced CVE. Male sex (hazard ratio 2.1; 95% confidence interval 1.13!3.66, p<0.01), hypertension (2.8;1.4–5.2; p<0.01), ischemic heart disease (6.8;3.5!13.2; p<0.0001), family history of CVD (2.1;1.1–3.9, p<0.05) and lipoprotein(a) level ≥30 mg/dl (2.8;1.1–7.7; p<0.05) were predictors of CVE. In FH patients the level of TC decreased by 19%, LDL!C by 25% (p<0.001 for both). Data on 72 VHR patients (60%) were obtained with follow!up duration of 19.7±5.8 months. No patient achieved the target LDL!C level of less than 1.4 mmol/L. Conclusion. Three!year follow!up of participants in the RENAISSANCE registry shows an enhanced adherence to hypolipidemic therapy. In FH patients the increased risk of new CVE is associated with male sex, hypertension, CHD, family history of CVD and lipoprotein(a) level ≥30 mg/dl

Aim. Russian multicenter register of familial hypercholesterolemia (FH) was transformed into Register of patients with FH and very high cardiovascular risk with insufficient effect of hypolipidemic therapy (RENESSANS Registry) in 2017 The aim of RENESSANS was maximal inclusion of patients not only with FH, but also those with atherosclerotic cardiovascular diseases (CVD), who did not achieve targeted level of low density lipoprotein cholesterol (LDL-C) using hypolipidemic drug therapy.Material and methods. The RENESSANS Registry is an open, national, observing study that includes patients with definite and probable (according to Dutch lipid clinic network and Simon Broome Registry criteria) heterozygous and homozygous FH, as well as patients of very high cardiovascular risk. There were designed two register forms: for patients with FH and for very high cardiovascular risk patients. Doctors filled out forms in paper and electronic variants. They took into consideration the risk factors of atherosclerosis and anamnesis of CVD, adherence to diet and hypolipidemic therapy. Concentrations of total cholesterol (TC), triglycerides (TG), high density lipoprotein cholesterol (HDL-C) were measured in blood serum in all centers. LDL-C level was defined according to Friedewald formula: LDL-C=TC-HDL-C-TG/2,2 (mmol/l).Results. The Registry consisted of 1208 FH patients and 497 patients with very high risk (average age 54±13 and 61±8, respectively, 37% men). Baseline levels of lipids were 9,4±2,3 and 6,9±1,5 mmol/l for TC, 6,6±2,1 and 4,5±1,3 mmol/l for LDL-C, respectively. The frequency of hypolipidemic therapy in both groups is 70%, while targeted level of LDL-C was achieved extremely rarely.Conclusion. The results show insufficient adherence and low effectiveness of standard hypolipidemic therapy both in patients with FH and very high cardiovascular risk. PCSK9 inhibitors are recommended for resistant hypercholesterolemia treatment. The RENESSANS Registry allows to improve FH diagnostics, to assess treatment effectiveness and choose patients who need treatment with PCSK9 inhibitors.