Malignant hyperthermia susceptibility. A light and electron microscopic study of endomyocardial biopsy specimens from nine patients.
Cardiomyopathy develops in some individuals who have a susceptibility to malignant hyperthermia. We studied right ventricular endomyocardial biopsy specimens from nine patients defined as having this disorder because of positive caffeine contracture tests on skeletal muscle biopsy specimens. Three patients had clinical evidence of cardiomyopathy and six did not. Light microscopy showed cytoplasmic contraction bands, perinuclear clearing, and a mild to moderate variation in myocyte and nuclear size. Ultrastructurally cytoplasmic contraction bands were associated with cardiac villi, myofiberlysis, and myofibrillolysis. These changes and occasional breaks in the sarcolemma were regarded as artefacts of the biopsy procedure. Megamitochondriosis with accompanying degenerative changes in the mitochondria were also seen and probably indicate increased cell metabolism; vacuolation of the cytoplasm was regarded as an "aging" phenomenon. Thus, the biopsy specimens were abnormal, but the changes were artefactual or nonspecific and were not unique to this group of patients. Biopsy did not provide a morphological explanation for abnormal cardiac function.
D000293:Adolescent; D000328:Adult; D002110:Caffeine / Q000009:adverse effects; D009202:Cardiomyopathies / Q000150:complications; D005260:Female; D006221:Halothane / Q000009:adverse effects; D006331:Heart Diseases / Q000235:genetics; D006801:Humans; D008297:Male; D008305:Malignant Hyperthermia / Q000150:complications* / Q000473:pathology*; D008854:Microscopy, Electron; D008875:Middle Aged; D009200:Myocardial Contraction / Q000187:drug effects; D009206:Myocardium / Q000473:pathology
D002110:Caffeine; D006221:Halothane