OA
Archives of Pediatrics and Child Health
Arrythmogenic Left Ventricle Induced By Desmoplakin Mutation
Authors: Manal Mohammed Helmy Nabo*
Publication Year: 2026
Abstract
Desmoplakin (DSP) is an essential component of the cardiac desmosome and contributes to mechanical coupling between cardiomyocytes.
Pathogenic variants in DSP are associated with a distinctive form of arrhythmogenic cardiomyopathy characterized by predominant left ventricular involvement, myocardial fibrosis, ventricular arrhythmia and progressive ventricular dysfunction [1–3].
The patient will presented by a variant of presentation.
But, the most important thing is the early detection as the permanent damage of the cardimyocyte is irreversible and need further treatment. The end result of untreated cases will be long life anti-failure and anti arrhythmic medication.