Atrioventricular block pathology in cardiomyopathy by desmin deposition.
Generally, restrictive cardiomyopathy due to desmin deposition is characterized by restriction to ventricular diastolic filling and different degrees of atrioventricular block (AVB). In this report, we describe the pathological changes of the cardiac conduction system related to AVB. The sinus node, the compact node, and the penetrating bundle (bundle of His) had no abnormalities, however, there was extensive fibrosis of the terminal portions of the branching bundle and the beginning of the left and right bundles at the top of the ventricular septum. The pathogenesis of this fibrous replacement is probably the same that leads to extensive fibrosis of the working ventricular myocardium, and remains to be elucidated.
Instituto do Coração, Hospital das Clínicas, FMUSP, São Paulo, Brazil. anpluiz@incor.usp.br
Luiz Alberto
LA
Benvenuti
Vera Dermarchi
VD
Aiello
Breno Alencar Araripe
BA
Falcão
Silvia Gelás
SG
Lage
D000293:Adolescent; D000328:Adult; D054537:Atrioventricular Block / Q000209:etiology* / Q000473:pathology; D002313:Cardiomyopathy, Restrictive / Q000150:complications* / Q000473:pathology; D003893:Desmin / Q000378:metabolism*; D017809:Fatal Outcome; D005355:Fibrosis; D006329:Heart Conduction System / Q000473:pathology*; D006801:Humans; D008297:Male
D003893:Desmin