Pédiatre – Cardiologue Huderf
2021-2022, 2022-2023, 2023-2024: "Bourse Claude Fickers"
Anatomy and structure of congenital heart diseases using μCT: example of congenitally corrected transposition of the great arteries and vascular malpositions
: Laboratory of Pediatric Research and Laboratory of Translational Research, Université Libre de Bruxelles (ULB), Brussels, Belgium and the Laboratory of Embryology and genetics of malformations, Institut Imagine, Paris, France
Congenital heart defects are among the most common birth defects, it is estimated that almost 1 in 100 children are born with a congenital heart defect. In the majority of cases, the cause of this anomaly is unknown (unrelated to genetic heritage) and the diagnosis is made before birth or during the first weeks of life. Congenitally corrected transposition of the great arteries is a rare congenital heart disease, its embryological mechanisms are still unknown to this day. This is a complex malformation whose management still presents real challenges today, including right ventricular failure which worsens over time. The goal of our research is to seek a better understanding of this heart defect and the mechanisms behind this right ventricular failure. In order to possibly suggest improvement concerning its management.
During a first work, we analyzed the anatomy of anatomical specimens with congenitall corrected transposition of the great arteries, and more precisely of their right ventricle. We compared this anatomy to other congenital heart defects (transposition of the great arteries) and normal hearts. We found differences in certain anatomical landmarks (septal band), although in both cases there are similarities (the vessels are parallel). This raises the hypothesis of different transposition mechanisms, and raises the question of the role of the inversion of the ventricles (in congenitally corrected transposition of the great arteries).
Our goal is to answer these questions by describing and analyzing the orientation of myocardial fibers in congenitally corrected transposition of the great arteries, transposition of the great arteries and normal hearts using μ-CT. More concretely, this work should make it possible, by modeling the myocardial and myofibrillary architecture of vascular malpositions, to optimize surgical repair techniques for these complex heart diseases, and also to optimize resynchronization techniques in systemic right ventricular heart disease, heart disease for which the management strategy is still far from optimal and well codified.