Abstrakt

Úvod: Koenzým Q10 (CoQ₁₀) zohráva kľúčovú úlohu pri syntéze adenozíntrifosfátu v dýchacom reťazci mitochondrií a súčasne pôsobí ako silný antioxidant. Jeho znížené hladiny boli potvrdené pri niektorých kardiovaskulárnych ochoreniach. U pacientov s Ebsteinovou anomáliou (EA) jeho koncetrácia doteraz nebola skúmaná.

Metódy: Realizovaná bola prierezová štúdia hodnotiaca koncentráciu CoQ₁₀, a-tokoferolu (aT), g-tokoferolu (gT), b-karoténu a produktov peroxidácie lipidov u pacientov s EA a zdravej kontrolnej skupiny v trombocytoch (PLT) a v plazme (PL). U pacientov s EA bola realizovaná magnetická rezonancia srdca (CMR). Hladiny antioxidantov boli porovnané medzi pacientmi s EA a kontrolnou skupinou. V skupine EA bola vykonaná podskupinová analýza na základe indexovaného objemu pravého komory (RV) v diastole (RVEDVi) podľa CMR.

Výsledky: Zaradených bolo 14 dospelých pacientov s EA a 18 zdravých kontrol. Priemerný vek v skupine EA bol 56 rokov, BMI 24,2 kg/m² a zastúpenie NYHA: 35,7/50/14,3/0 % (I/II/III/IV). CMR potvrdila diagnózu EA s priemerným posunom septálneho cípu o 16,1±6,73 mm/m². Koncentrácia CoQ₁₀ a gT v PLT boli signifikantne nižšie v skupine EA v porovnaní s kontrolnou skupinou (81,50 ± 26,70 vs 58,20 ± 28,00 pmol/10^-9, P = 0,02 a 157,00 ± 169,00 vs 62,0 ± 57,50 pmol/10^{-9 PLT}, P = 0,01), koncentrácie aT boli vyššie (4980,00 ± 3214,00 vs 7772,00 ± 3202,00 pmol/10^{-9 PLT}, P = 0,027). Koncentrácia CoQ₁₀ v PL aj PLT pozitívne korelovala s RVEDVi (R = 0,464, P = 0,045 a R = 0,476, P = 0,047), koncentrácia aT v PL korelovala negatívne (R = -0,460, P = 0,049). Analýza podskupín potvrdila signifikantne nižšie hladiny CoQ₁₀ v PL aj PLT u pacientov s nedilatovanou RV v porovní s pacientmi s dilatovanou RV (0,32 ± 0,11 vs 0,50 ± 1,19 µmol/L ^PL, P = 0,046 a 43,50 ± 13,77 vs 72,80 ± 31,73 pmol/10^{-9 PLT}, P = 0,044), kocentrácie iných antioxidantov neboli významne odlišné medzi skupinami.

Záver: Uvedená štúdia potvrdila signifikantne znížené hladiny CoQ₁₀, gT a zvýšené hladiny aT u pacientov s EA v porovnaní so zdravými kontrolami. Objem RV pozitívne koreloval s CoQ₁₀ a nagatívne s aT. U pacientov s nedilatovanou RV boli potvrdené najnižšie hladiny CoQ₁₀. Koncentrácie antioxidantov môžu súvisieť s progresiou EA, pričom deficit CoQ₁₀ môže odrážať primárne ochorenie myokardu RV (Tab. 5, Fig. 3, Ref. 26). Text v PDF www.lekarsky.herba.sk.

KĽÚČOVÉ SLOVÁ: koenzým Q₁₀, Ebsteinova anomália, antioxidanty, kardiomyopatia.

Lek Obz 2025, 74 (4): 132-140

The first evidence for a deficiency of coenzyme Q10 in patients with ebstein anomaly

Abstract

Background: Coenzyme Q₁₀ (CoQ₁₀) plays a crucial role in adenosine triphosphate synthesis within the mitochondrial respiratory chain and acts as a potent antioxidant, mitigating reactive oxygen species. Its deficiency has been documented in several cardiac disorders. However, coenzyme Q₁₀ levels in patients with Ebstein‘s anomaly (EA) remain unexplored.

Methods: This cross-sectional study evaluated endogenous CoQ₁₀ levels, as well as concentrations of a-tocopherol (aT), g-tocopherol (gT), b-carotene, and lipid peroxidation products in plasma (PL) and thrombocytes (PLT), in both EA patients and a control healthy group. Cardiac magnetic resonance imaging (CMR) was performed exclusively in the EA group. Antioxidant levels were compared between EA patients and healthy controls, while within the EA group, additional comparisons were made based on indexed CMR-derived right ventricular (RV) end-diastolic volume (RVEDVi).

Results: This study involved 14 adult patients with confirmed EA and 18 healthy controls. The EA group had a mean age of 56 years, a mean BMI of 24.2 kg/m², and the following NYHA classifications: 35.7%/50%/14.3%/0% (I/II/III/IV). CMR confirmed the EA diagnosis with septal leaflet displacement of 16,1±6,73mm/m². CoQ₁₀ and gT levels in platelets were significantly lower in the EA group (81.50±26.70 vs 58.20±28.00pmol/10^{-9 PLT}, P=0.02 and 157.00±169.00 vs 62.0±57.50pmol/10^{-9 PLT}, P=0.01), while aT was higher (4980.00±3214.00 vs 7772.00±3202.00pmol/10^{-9 PLT}, P=0.027). CoQ₁₀ in PL and PLT showed moderate positive correlations with CMR-derived RVEDVi (R=0.464, P=0.045 and R=0.476, P=0.047), while aT in plasma correlated negatively (R=-0.460, P=0.049). Subgroup analysis showed significantly lower CoQ₁₀ levels in both PL and PLT in the non-dilated RV group compared to the dilated RV (0.32±0.11 vs 0.50±1.19µmol/L^PL, P=0.046 and 43.50±13.77 vs 72.80±31.73pmol/10^{-9 PLT}, P=0.044), with no other antioxidant differences between groups.

Conclusion: This study identified significantly reduced levels of CoQ₁₀, gT and increased  aT in patients with EA compared to healthy controls. RVEDVi showed positive correlation with CoQ₁₀ and negative correlation with aT. The lowest CoQ₁₀ levels were observed in patients with EA and non-dilated RV. Antioxidants may be linked to EA disease progression, while CoQ₁₀ deficiency may reflect intrinsic myocardial abnormalities (Tab. 5, Fig. 3, Ref. 26). Text in PDF www.lekarsky.herba.sk.

KEY WORDS: coenzyme Q₁₀, Ebstein anomaly, antioxidants, cardiomyopathy.

Lek Obz 2025, 74 (4): 132-140

Filip KLAUČO ¹, Zuzana SUMBALOVÁ ², Tereza HLAVATÁ ¹, Monika KALDARÁROVÁ ³, Anna GVOZDJÁKOVÁ ⁴, Iveta ŠIMKOVÁ ¹

¹ Department of Cardiology and Angiology, Slovak Medical University and National Institute of Cardiovascular Diseases, Pod Krásnou hôrkou 1, 833 48 Bratislava, Slovakia, head prof. I. Šimková, MD, PhD.
² Institute of Medical Chemistry, Biochemistry and Clinical Biochemistry, Faculty of Medicine, Comenius University Bratislava, Sasinkova 2, 811 08 Bratislava, Slovakia Head: prof. Ladislav Turecký, MD, PhD
³ Children‘s Cardiac Center, National Institute of Cardiovascular Diseases, Pod Krásnou hôrkou 1, 833 48 Bratislava, Slovakia. Head: Peter Tittel, MD, PhD
⁴ Slovak Medical University, Faculty of Medicine, Limbová 12, 833 03 Bratislava