Abstract
BackgroundVentricular 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. MethodsUsing 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. ResultsIn 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. ConclusionThe 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.
Highlights
Left ventricular noncompaction or hypertrabeculation is a cardiomyopathy characterized by excessive trabeculations and is associated with a high risk of heart failure and sudden cardiac death [1,2]
We compare normal and noncompacted fetal human hearts and show the Purkinje myocardium is not excessive in noncompaction. This finding is surprising if one expects noncompaction to develop from excessive embryonic trabeculations, but is not surprising given mouse models of noncompaction [10,11] showing the compact wall growing into the ventricular lumen in a trabecular fashion
Selected cases included 3 hearts (NC1, 2 and 3) with a diagnosis of ventricular noncompaction based on the ratio of left ventricular trabeculated versus compacted myocardium greater than 2.3:1, as was measured on a haematoxylin and eosin (HE) stained section of the tissue blocks
Summary
Ventricular noncompaction is characterized by excessive trabeculations and is associated with heart failure. Results: In the embryo, the ventricular trabeculations strongly expressed ANF/NPPA whereas the compact wall did not. In noncompaction and control fetal hearts alike, 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. We propose the lesion results from the compact wall growing into the ventricular lumen in a trabecular fashion
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