Bjarke Jensen1, Allard C van der Wal2, Antoon F M Moorman3, Vincent M Christoffels3. 1. Department of Anatomy, Embryology & Physiology, Academic Medical Center, University of Amsterdam, The Netherlands. Electronic address: b.jensen@amc.uva.nl. 2. Department of Pathology, Academic Medical Center, University of Amsterdam, The Netherlands. 3. Department of Anatomy, Embryology & Physiology, Academic Medical Center, University of Amsterdam, The Netherlands.
Abstract
BACKGROUND: Ventricular noncompaction is characterized by excessive trabeculations and is associated with heart failure. The lesion is hypothesized to result from failed compaction and thus retention of embryonic trabeculations. Here, we assess for the first time the identity of trabeculations in noncompaction to test whether noncompacted hearts show retention of embryonic trabeculations. METHODS: Using immunohistochemistry, we analyzed cardiac sections of the heart of a control embryo, 3 cases of fetal noncompaction (a set of twins and an unrelated fetus) and 3 fetal hearts without noncompaction. RESULTS: In the embryo, the ventricular trabeculations strongly expressed ANF/NPPA whereas the compact wall did not. In the noncompaction hearts, trabeculations constituted an excessively thick layer. In noncompaction and control fetal hearts alike, however, only a miniscule subset of sub-endocardial myocardium of the trabeculations most proximal to the central ventricular lumen exhibited strong expression of ANF/NPPA, representing Purkinje myocardium. The trabeculations of both fetal control and noncompaction hearts were ANF-negative and orders of magnitude wider than those of the embryo. Both the compact and noncompaction trabeculated myocardium were rich in coronary vasculature. Like embryonic trabeculations, the ANF+ Purkinje myocardium had little if any vasculature. CONCLUSION: The excessive trabeculations in noncompaction do not have the embryonic identity and noncompaction is probably not the result of failed compaction. We propose the lesion results from the compact wall growing into the ventricular lumen in a trabecular fashion.
BACKGROUND: Ventricular noncompaction is characterized by excessive trabeculations and is associated with heart failure. The lesion is hypothesized to result from failed compaction and thus retention of embryonic trabeculations. Here, we assess for the first time the identity of trabeculations in noncompaction to test whether noncompacted hearts show retention of embryonic trabeculations. METHODS: Using immunohistochemistry, we analyzed cardiac sections of the heart of a control embryo, 3 cases of fetal noncompaction (a set of twins and an unrelated fetus) and 3 fetal hearts without noncompaction. RESULTS: In the embryo, the ventricular trabeculations strongly expressed ANF/NPPA whereas the compact wall did not. In the noncompaction hearts, trabeculations constituted an excessively thick layer. In noncompaction and control fetal hearts alike, however, only a miniscule subset of sub-endocardial myocardium of the trabeculations most proximal to the central ventricular lumen exhibited strong expression of ANF/NPPA, representing Purkinje myocardium. The trabeculations of both fetal control and noncompaction hearts were ANF-negative and orders of magnitude wider than those of the embryo. Both the compact and noncompaction trabeculated myocardium were rich in coronary vasculature. Like embryonic trabeculations, the ANF+ Purkinje myocardium had little if any vasculature. CONCLUSION: The excessive trabeculations in noncompaction do not have the embryonic identity and noncompaction is probably not the result of failed compaction. We propose the lesion results from the compact wall growing into the ventricular lumen in a trabecular fashion.
Authors: Madhav Mantri; Gaetano J Scuderi; Roozbeh Abedini-Nassab; Michael F Z Wang; David McKellar; Hao Shi; Benjamin Grodner; Jonathan T Butcher; Iwijn De Vlaminck Journal: Nat Commun Date: 2021-03-19 Impact factor: 14.919
Authors: Bjarke Jensen; Bastiaan J Boukens; Dane A Crossley; Justin Conner; Rajiv A Mohan; Karel van Duijvenboden; Alex V Postma; Christopher R Gloschat; Ruth M Elsey; David Sedmera; Igor R Efimov; Vincent M Christoffels Journal: Elife Date: 2018-03-22 Impact factor: 8.140
Authors: Hanne C E Riekerk; Bram F Coolen; Gustav J Strijkers; Allard C van der Wal; Steffen E Petersen; Mary N Sheppard; Roelof-Jan Oostra; Vincent M Christoffels; Bjarke Jensen Journal: J Anat Date: 2021-09-26 Impact factor: 2.610