What question did this study set out to answer?

To evaluate coenzyme Q10 levels in patients with Ebstein anomaly and compare with healthy controls.

February 8, 2026

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

FKF KlaucoSlovak Medical University ZSZ SumbalovaComenius University Bratislava THTereza HlavataNational Institute of Cardiovascular Diseases

Key Points

To evaluate coenzyme Q10 levels in patients with Ebstein anomaly and compare with healthy controls.
Cross-sectional examination of CoQ10 and antioxidant levels in plasma and platelets.
Conducted cardiac magnetic resonance imaging in patients with Ebstein anomaly.
Compared antioxidant levels between patients with Ebstein anomaly and healthy controls.
CoQ10 and γT levels were significantly lower in patients with Ebstein anomaly than in healthy controls.
αT levels were significantly higher in the Ebstein anomaly group.
CoQ10 showed a moderate positive correlation with right ventricular end-diastolic volume.
The lowest CoQ10 levels were observed in patients with non-dilated right ventricles.

Abstract

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 α-tocopherol (αT), γ-tocopherol (γT), β-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 γT 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 αT 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 αT 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/LPL, 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, γT and increased αT in patients with EA compared to healthy controls. RVEDVi showed positive correlation with CoQ10 and negative correlation with αT. 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

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