Arrhythmogenic right ventricular cardiomyopathy is a hereditary disorder characterized by  structural and functional abnormalities in the right ventricle causing ventricular arrhythmia.

What is the Pathology of Arrhythmogenic Right Ventricular Cardiomyopathy?

Arrhythmogenic right ventricular cardiomyopathy pathology consists of myocardial atrophy of the right ventricle with transmural fatty replacement resulting in electrical instability. 

Arrhythmogenic Right Ventricular Cardiomyopathy?

Arrhythmogenic right ventricular cardiomyopathy is an important cause of sudden cardiac death in 11% of young adults. It presents as palpitation, syncope, sudden cardiac death, atypical chest pain, and dyspnea. 

How is Arrhythmogenic Right Ventricular Cardiomyopathy Diagnosed?

Arrhythmogenic right ventricular cardiomyopathy diagnosis consists of electrocardiogram (inverted T-waves, epsilon waves), 2D echo, and 24-hr holter monitoring.  

How is Arrhythmogenic Right Ventricular Cardiomyopathy Treated?

Arrhythmogenic right ventricular cardiomyopathy treatment consists of lifestyle changes, pharmacologic therapy, catheter-based ablation, placement of an implantable cardioverter-defibrillator (ICD), and heart transplantation. 

What is the Prognosis of Arrhythmogenic Right Ventricular Cardiomyopathy?

Arrhythmogenic right ventricular cardiomyopathy prognosis is poor with mortality of 22% in young adult athletes. Poorer prognosis is seen in patients with left ventricular involvement.