A case of arrhythmogenic right ventricular dysplasia/cardiomyopathy with pneumothorax and degloving injury of the right upper limb

Abstract

Arrhythmogenic right ventricular dysplasia / cardiomyopathy (ARVD / C) is a rare genetic cardiomyopathy characterized by fibro-fatty replacement primarily of the right ventricular myocytes,and can present as any of 'concealed' disease, 'overt arrhythmia' followed by sudden cardiac death, isolated right heart failure or biventricular failure: mimicking dilated cardiomyopathy. We present the successful management of a 65 year old male patient with ARVD/C presenting to us with trauma and Cardiogenic shock for limb saving emergency surgery.

Keywords

• arrhythmogenic right ventricular dysplasia
• dilated cardiomyopathy
• plakophilin