title: [Myocardial remodeling in pediatric congenital cardiac diseases].
abstract: Myocardial remodeling in pediatric congenital cardiac diseases is related to myocardial dysfunction and increases morbidity and mortality. The complex mechanisms that characterize this state involve inflammatory-, growth- and death signaling. The studies we have conducted in infants with hemodynamic overload of the right ventricle, associated or not with hypoxemia, show that mechanical stress related to pressure overload of the right ventricle leads to myocardial expression of pro-inflammatory cytokines such as TNF-α, IL-1β et IL-6, mainly via the activation of p38MAPK signaling. Furthermore, hypoxemia induces activation of the transcription factor HIF-1 that, in turn, induces its target genes VEGF and eNOS. This might be interpreted as an adaptive response. Hypoxemia also contributes to stimulation of growth signaling as it induces CT-1 via the JAK/STAT pathway. The observation that hypoxemia is also associated with a higher degree of troponin T degradation might indicate a loss of the protective function of CT-1 to the myocardium in infants with cyanotic congenital cardiac diseases.
authors: M C
authors: MC
authors: Seghaye
mesh_terms: D002648:Child; D006330:Heart Defects, Congenital / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D006439:Hemodynamics; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D020257:Ventricular Remodeling