Arrhythmogenic Right Ventricular Dysplasia (ARVD), also known as arrhythmogenic cardiomyopathy, is an inherited cardiomyopathy. It is disproportionately highly represented in cases of unexplained death under anesthesia, but there are few published reports of the safe anaesthetic management of patients with ARVD. Arrhythmogenic right ventricular dysplasia is a form of genetic cardiomyopathy that is a well-recognised cause of ventricular tachycardia and sudden death, particularly in athletes and the young. ARVD is also one of the leading causes of death during anaesthesia. In one reported analysis of forensic autopsies, eighteen out of fifty perioperative deaths in ASA 1 patients undergoing surgical procedures, usually associated with extremely low mortality, have previously been found at autopsy to show histological features of ARVD. None of these patients had any prior cardiac history. Four of the patients died at induction of anaesthesia, nine during the surgical procedure and five within 2 hours after surgery was complete.
The estimated prevalence of ARVD in the general population is about 1 in 5,000. Young men are affected approximately three times more frequently than women, with a higher prevalence (up to 0.8%) reported in certain parts of Italy (Veneto) and Greece (Naxos Island). At a prevalence rate of 1 in 5,000, most anaesthetists will encounter patients with ARVD, in some of whom the diagnosis may not have been established. The histological demonstration of myocyte atrophy and transmural fibro-fatty replacement of right ventricular myocardium is typical for this condition. Immunohistochemistry for plakoglobin may also add to the accuracy of this diagnosis. Interestingly, in 70% of the cases the left ventricle is also involved. ARVD is caused by mutation in the desmosomal proteins Plakophillin 2 (PKP2), Desmoglein 2 (DSG2), Desmocollin 2 (DSC2), Desmoplakin (DSP), Plakoglobin (JUP), and others.