title: [Myocardial dysplasia of the right ventricle (Uhl's anomaly) as a possible cause of sudden, unexpected death].
abstract: Partial absence and fatty replacement of the myocardial compacta of the right ventricular wall was observed in five young male adults, four of whom died suddenly and unexpectedly and one in a car accident. In one of these cases there was only one coronary orifice. Four cases of the so-called Uhl anomaly had histological findings indicating dysmorphic myocardial cells associated with minimal lymphohistiocytic infiltrates within the fatty tissue. In one case, chronic destructive inflammation was a prominent feature. Marked dysmorphic alterations in the myocardial fibers suggested the occurrence of arrhythmic episodes as the main cause of sudden heart failure and death.
authors: Gerichtsmedizinisches Institut am Kantonsspital, St. Gallen, Schweiz.
authors: T
authors: T
authors: Sigrist
authors: W
authors: W
authors: Bär
authors: P
authors: P
authors: Zink
mesh_terms: D000273:Adipose Tissue / Q000473:pathology; D000293:Adolescent; D000328:Adult; D005117:Cardiac Complexes, Premature / Q000473:pathology; D009202:Cardiomyopathies / Q000151:congenital* / Q000473:pathology; D002423:Cause of Death; D003645:Death, Sudden / Q000473:pathology*; D003937:Diagnosis, Differential; D006333:Heart Failure / Q000473:pathology; D006352:Heart Ventricles / Q000002:abnormalities* / Q000473:pathology; D006801:Humans; D008297:Male
vernacular_title: Myokardiale Dysplasie der rechten Herzkammer (Uhl'sche Anomalie) als mögliche Ursache eines plötzlichen, unerwarteten Todes.