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Войти в исследования Pediatric research · 2026
A complementary proteomics and cardiac magnetic resonance approach to characterize right ventricular remodeling after tetralogy of Fallot repair.
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Jones Andrea L, Edwards Jonathan J, Fazelinia Hossein, Frank David, Clouthier Katie L, Patel Chandni, Spruce Lynn, Ding Hua, Sabella Julia, Avitabile Catherine M, Friedberg Mark K, Fogel Mark A, Ischiropoulos Harry, Reddy Sushma, Mercer-Rosa Laura
Аннотация
BACKGROUND: Patients with repaired tetralogy of Fallot (TOF) can develop right ventricular (RV) dilation and failure. We sought to utilize an untargeted serum proteomics approach to characterize the proteome signature associated with RV dilation in repaired TOF to improve the understanding of mechanistic drivers of RV remodeling.
METHODS: Patients with repaired TOF undergoing cardiac magnetic resonance imaging were recruited. Groups were based on indexed RV end-diastolic volume (RVEDVi). Serum proteomics identified differentially abundant proteins (p value < 0.05), and pathway enrichment identified functional pathways.
RESULTS: Fourteen TOF adolescents (median age 16 years; IQR 15, 18) were divided into Group 1 (n = 9; median RVEDVi 146 ml/m2; IQR 139, 164) and Group 2 (n = 5; median RVEDVi 114 ml/m2; IQR 104, 130). Group 1 had a higher indexed RV stroke volume (85 ml/m2 vs. 59 ml/m2, p = 0.023) with a similar RV ejection fraction. Group 1 had altered regulation of genes in multiple pathways, including glucose and fatty acid metabolism and antioxidant response.
CONCLUSION: Patients with repaired TOF and moderate-severe RV dilation have a distinct serum proteomic profile despite preserved ejection fraction, which may reflect alterations in fuel sources and oxidative stress. These hypothesis-generating findings are a first step in the integration of proteomics into clinical management.
IMPACT: An untargeted serum proteomics approach to identifying altered protein profiles associated with CMR-defined RV dilation is a novel approach to characterizing right ventricular remodeling after tetralogy of Fallot repair. RV dilation after repair of tetralogy of Fallot is associated with a specific proteomic profile suggesting adaptive and maladaptive remodeling. Identification of dysregulated proteins associated with RV remodeling provides insight into the mechanisms leading to ventricular failure after tetralogy of Fallot repair. With additional data from future larger studies, serum proteomics evaluation may assist in prognostication and clinical decision making in patients after tetralogy of Fallot repair.
METHODS: Patients with repaired TOF undergoing cardiac magnetic resonance imaging were recruited. Groups were based on indexed RV end-diastolic volume (RVEDVi). Serum proteomics identified differentially abundant proteins (p value < 0.05), and pathway enrichment identified functional pathways.
RESULTS: Fourteen TOF adolescents (median age 16 years; IQR 15, 18) were divided into Group 1 (n = 9; median RVEDVi 146 ml/m2; IQR 139, 164) and Group 2 (n = 5; median RVEDVi 114 ml/m2; IQR 104, 130). Group 1 had a higher indexed RV stroke volume (85 ml/m2 vs. 59 ml/m2, p = 0.023) with a similar RV ejection fraction. Group 1 had altered regulation of genes in multiple pathways, including glucose and fatty acid metabolism and antioxidant response.
CONCLUSION: Patients with repaired TOF and moderate-severe RV dilation have a distinct serum proteomic profile despite preserved ejection fraction, which may reflect alterations in fuel sources and oxidative stress. These hypothesis-generating findings are a first step in the integration of proteomics into clinical management.
IMPACT: An untargeted serum proteomics approach to identifying altered protein profiles associated with CMR-defined RV dilation is a novel approach to characterizing right ventricular remodeling after tetralogy of Fallot repair. RV dilation after repair of tetralogy of Fallot is associated with a specific proteomic profile suggesting adaptive and maladaptive remodeling. Identification of dysregulated proteins associated with RV remodeling provides insight into the mechanisms leading to ventricular failure after tetralogy of Fallot repair. With additional data from future larger studies, serum proteomics evaluation may assist in prognostication and clinical decision making in patients after tetralogy of Fallot repair.