Primary Noncompaction of the Ventricular Myocardium from the Morphogenetic Standpoint |
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Authors: | U Bartram J Bauer D Schranz |
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Institution: | Department of Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital Giessen, Feulgenstrasse 12, 35390 Giessen, Germany. ulrike.bartram@paediat.med.uni-giessen.de |
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Abstract: | This review compiles the current knowledge of normal and abnormal myocardial morphogenesis to facilitate an unambiguous diagnosis
of primary myocardial noncompaction. During the early stages of development, the formation of trabeculae with the resulting
increase in myocardial surface is a adaptation of the rapidly growing heart to improve nourishment by exchange diffusion from
the cardiac lumen. Once the coronary vasculature has developed, the switch to cardiac nutrient supply through active circulation
from the subepicardial space is paralleled by gradual compaction of the myocardial trabeculae. This results in a decrease
of the inner, trabeculated myocardial layer with a parallel increase in thickness of the outer, compact myocardial layer.
Similar to the direction of coronary arterial development, this process proceeds from the epicardium toward the endocardium
and from the base of the heart to the apex. Based on developmental data, congenital myocardial noncompaction represents a
failure of normal embryonic myocardial maturation. The time of arrest of this process will determine the extension of myocardial
noncompaction within the ventricle. Whereas disturbances of myocardial microcirculation are frequent in these hearts, direct
communications between the myocardial cavity and the coronary arteries (sinusoids) do not belong to this morphogenetic entity. |
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Keywords: | Myocardial noncompaction Heart development Cardiac morphogenesis Coronary arteries |
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