Noncompaction in Duchenne muscular dystrophy: frustrated attempt to create a compensatory left ventricle?
The histological workup of the myocardium of a patient with Duchenne muscular dystrophy and left ventricular hypertrabeculation/noncompaction (LVHT) revealed an extremely thin left ventricular wall and a noncompacted layer double in size compared to the compacted layer. Within the compacted layer islets of fibrous tissue predominated, surrounded by areas of myxoid appearance hardly producing collagen, and occasionally normal or dystrophic cardiomyocytes. The noncompacted layer consisted largely of intact cardiomyocytes rarely intermingled with collagen-producing, fibrous tissue. This variant appearance of the compacted and noncompacted layer was found in all areas of noncompaction. These histopathological findings suggest that LVHT represents a compensatory attempt to overcome the failing compacted but dystrophic myocardium.
Finsterer
Josef
J
Department of Neurology, Krankenanstalt Rudolfstiftung, Vienna, Austria. duarte@aonmail.at
Stöllberger
Claudia
C
Feichtinger
Hans
H
D000328:Adult; D001344:Autopsy; D009202:Cardiomyopathies / Q000473:pathology*; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000473:pathology*; D018487:Ventricular Dysfunction, Left / Q000473:pathology*