Perioperative management of severe head injury in a patient with arrhythmogenic right ventricular dysplasia.

ARRHYTHMOGENIC right ventricular dysplasia (ARVD) is a recently described form of cardiomyopathy characterized by the occurrence of ventricular tachycardia of right ventricular origin. ARVD is characterized by fibrofatty replacement of the right myocardium, linked in part to both apoptotic cell death and patchy myocarditis. ARVD is an important cause of sudden arrhythmic death in young patients, especially during physical exercise, and severe forms of the disease may also lead to congestive heart failure. Diagnosis is based on clinical criteria and results from specialized testing—in particular, contrast ventriculography. Antiarrhythmic drug therapy is the first step of treatment, but the severity of ventricular tachycardia (VT) may necessitate surgical or catheter ablative techniques or an implantable defibrillator. Specific problems that may be encountered during the perioperative period have not been described. We report the specific management of severe head trauma in a patient with known ARVD.