ArticleProgress in pediatric cardiology
Long-chain fatty acid utilization in Eisenmenger syndrome.
Article in Progress in pediatric cardiology. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Authors and funding
7 authors.
Funding
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 ( Conclusions: Our preliminary data in ES differs importantly and unexpectedly from left ventricular and RV failure, in that circulating LCFA utilization appears to be increased. Further, TCA cycle activity is increased in ES, supporting that mechanisms which preserve or enhance LCFA utilization may ultimately preferentially support FAO for energy production. Further study of the impact of LCFA utilization on RV function in ES and other cohorts will be important to determine the role that metabolic substrates may play in long-term preservation of heart function in unique groups.
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