Key result
Ebstein anomaly meeting surgical indications linked to ~2.6-fold higher platelet Complex I activity.
Why the study?
Identifying the optimal timing for surgical intervention before irreversible myocardial damage occurs in Ebstein anomaly remains challenging. Whether platelet mitochondrial bioenergetics can serve as a translational biomarker for disease severity and right ventricular dysfunction in these patients was investigated.
Does platelet mitochondrial bioenergetic profiling serve as a translational biomarker for disease severity and right ventricular dysfunction in patients with Ebstein anomaly?
Case-Control (n=32)
Does platelet mitochondrial bioenergetic profiling serve as a translational biomarker for disease severity and right ventricular dysfunction in patients with Ebstein anomaly?
Platelet mitochondrial bioenergetic profiling reveals hyperactivated respiration and antioxidant depletion in severe Ebstein anomaly, offering a promising novel biomarker for risk stratification and timing of surgical intervention.
May inform severity assessment in Ebstein anomaly; leaves open prospective validation before clinical use.
Background Chronic right ventricular (RV) volume overload due to primary tricuspid regurgitation in Ebstein anomaly (EA) drives progressive dilatation and pathological myocardial remodeling. Identifying the optimal timing for surgical intervention before irreversible myocardial damage occurs remains challenging. Since platelets reflect systemic metabolic shifts, this study investigates whether platelet mitochondrial bioenergetics can serve as a translational biomarker for disease severity and RV dysfunction in EA patients. Methods Analysed were isolated platelets from 14 EA patients and 18 healthy controls. The patient cohort was stratified based on the 2020 ESC guidelines into two groups: EA-0 (no current indication for surgery) and EA-1 (meeting Class Ia or IIb indications for surgical intervention). High-resolution respirometry was utilized to evaluate oxidative phosphorylation (OXPHOS), fatty acid oxidation (FAO), and electron transport capacity. Additionally, endogenous antioxidant levels (CoQ10 and γ-tocopherol) were quantified. Results Complex I (CI)-linked respiration and FAO-linked OXPHOS were moderately elevated in the EA-0 cohort (120% and 122% of controls, respectively) but exhibited a marked, significant hyperactivation in the EA-1 cohort (CI: 259%; FAO: 197%). This increased oxygen consumption in EA-1 was accompanied by severe depletion of endogenous antioxidants, specifically CoQ10 (59% of controls) and γ-tocopherol (23% of controls). Conclusion Mitochondrial bioenergetics revealed compensatory metabolic reprogramming that strongly correlated with clinical severity. The pronounced hyperactivation of CI-linked respiration, combined with severe CoQ10 deficiency in platelets, reflects a systemic maladaptive response and reductive stress driven by chronic RV overload. This accessible bioenergetic profile provides a promising novel biomarker for personalized risk stratification, offering valuable mechanistic insights to guide the optimal timing of surgical intervention in EA.
No takes yet. Share an insight, caveat, or question.
Klauco et al. (2026) conducted a case-control in Ebstein anomaly (n=32). Ebstein anomaly (stratified by surgical indication into EA-0 and EA-1) vs. Healthy controls was evaluated on Platelet mitochondrial bioenergetics (Complex I-linked respiration and FAO-linked OXPHOS) and endogenous antioxidant levels (CoQ10 and γ-tocopherol) relative to controls. Ebstein anomaly meeting surgical indications showed marked platelet Complex I (259% of controls) and FAO (197%) hyperactivation alongside CoQ10 (59%) and γ-tocopherol (23%) depletion.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:
More from European Heart Journal