desmin – ABC CARDIOL

ABC

Case Report

Atrioventricular block pathology in cardiomyopathy by desmin deposition

Luiz Alberto Benvenuti et al. Vera Dermarchi Aiello Breno Alencar Araripe Falcão Silvia Gelás Lage

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.

Read Full Article

ABC

Case Report

Desmin-related restrictive cardiomyopathy

Christiano Pereira Silva et al. Fernando Bacal Luiz Alberto Benvenuti Edimar Alcides Bocchi

Restrictive cardiomyopathies may have different etiologies, among which we can point out storage diseases by accumulation of different materials such as desmin. Desminopathies are uncommon diseases that progress with conduction abnormalities, peripheral myopathies, and ventricular dysfunction. The present report describes a patient with complete atrioventricular block as the initial event; he later developed skeletal muscle alterations and heart failure. The investigation led to the diagnosis of restrictive cardiomyopathy due to desmin accumulation.

Read Full Article

Atrioventricular block pathology in cardiomyopathy by desmin deposition

Desmin-related restrictive cardiomyopathy

Electronic Document Format (Vancouver)

Electronic Document Format (ABNT)

Electronic Document Format (APA)

Electronic Document Format (ISO)

Electronic Document Format (Vancouver)

Electronic Document Format (ABNT)

Electronic Document Format (APA)

Electronic Document Format (ISO)