Case report: DSP truncation variant p. R1951X leads to arrhythmogenic left ventricular cardiomyopathy.

ABSTRACT:

Background

Standardized diagnostic criteria for arrhythmogenic left ventricular cardiomyopathy (ALVC) have been recently proposed. The criteria emphasize structural left ventricle (LV) myocardial change on contrast-enhanced imaging and require the identification of gene variants associated with arrhythmogenic cardiomyopathy.

Case summary

A 21-year-old man presented for evaluation of exertional syncope and was found to have monomorphic ventricular tachycardia (VT) and an episode of polymorphic VT that degenerated to ventricular fibrillatory cardiac arrest. Documented premature ventricular contractions were of left bundle branch block, inferior axis morphology. Ventricular arrhythmias were successfully suppressed with β-blockade, amiodarone, and lidocaine, and a subcutaneous i

SUBMITTER: Chen V 

PROVIDER: S-EPMC9026210 | biostudies-literature | 2022 Mar

REPOSITORIES: biostudies-literature