Long-chain fatty acid utilization in Eisenmenger syndrome
Abstract
Background: Morbidity and mortality in idiopathic pulmonary arterial hypertension (iPAH) are linked to right ventricular failure (RVF), a condition characterized by a metabolic shift from fatty acid oxidation (FAO) to glycolysis. Eisenmenger Syndrome (ES), a unique form of PAH, is associated with a mortality paradox, yet the mechanism for better survival in this condition remains poorly understood.
Objectives: We postulated that beneficial changes in long chain fatty acid (LCFA) utilization may support right ventricular (RV) adaptations in ES, and therefore ssupport favorable RV function and improved morbidity and mortality when compared to iPAH.
Methods: We examined clinical and metabolomic data to identify potential mechanistic clues about RV function and morbidity/mortality in controls, iPAH, and ES subjects.
Results: In a small cohort with iPAH (n = 3) or ES (n = 3) clinical data demonstrated worse hemodynamic disease in ES (mean pulmonary artery/mPA pressure 90 ± 6 vs. 50 ± 4 mmHg, p p ≤ 0.05 and lower brain natriuretic peptide levels 11 ± 6 pg/dL vs. 71 ± 18 pg/dL, p < 0.01). Metabolomic analysis demonstrated that 97 circulating lipids were significantly different between iPAH and ES. Plasma concentration of LCFAs (Stearate, Palmitate, Oleate, Lineolate) were significantly reduced in ES compared to iPAH, and all but Lineolate were also reduced when compared to non-PAH control samples, suggesting there may be an advantageous compensatory increase in LCFA utilization…
How strong is the evidence?
Evidence Hub reads this as a cohort study. Its evidence level is 3 of 5 (Moderate · trial / cohort / secondary analysis). Trials without a large randomized design, cohorts and secondary analyses give moderate evidence.
Read the paper
Related studies
- eggNOG 5.0: a hierarchical, functionally and phylogenetically annotated orthology resource based on 5090 organisms and 2502 viruses
- Online Survival Analysis Software to Assess the Prognostic Value of Biomarkers Using Transcriptomic Data in Non-Small-Cell Lung Cancer
- OptiType: precision HLA typing from next-generation sequencing data
- Nicotinic Cholinergic System and COVID-19: In Silico Identification of an Interaction between SARS-CoV-2 and Nicotinic Receptors with Potential Therapeutic Targeting Implications
- Saccharomyces cerevisiae cells have three Omega class glutathione S-transferases acting as 1-Cys thiol transferases
- Hemolysis at low blood flow rates: in-vitro and in-silico evaluation of a centrifugal blood pump
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.