[Anesthetic management of patients with arrhythmogenic right ventricular cardiomyopathy].

Arrhythmogenic right ventricular cardiomyopathy is a genetic cardiomyopathy characterized by replacement of right ventricular myocardium by fibrofatty infiltrates, leading to significant ventricular arrhythmias with sudden death and right ventricular dysfunction. Elective operations should be postponed, until the arrhythmias and myocardial function are well tolerated. There has been no guideline on the anesthetic management of this serious, despite rare, disease and there are a few reports of the patients undergoing operation under either general or regional anesthesia. The most important issue of the anesthetic management is to avoid excessive sympathetic stimulation, especially beta stimulation which can easily induce life-threatening arrhythmias. Also, it is better to avoid adrenalin as an adjunct to the local anesthetics. Anesthetic maintenance was performed with volatile anesthetics except halothane, opiates, muscle relaxants except pancuronium and intravenous anesthetics including propofol, ketamine and benzodiazepines. Invasive monitoring of arterial blood pressure and central venous pressure is recommended and transesophageal echocardiography, if available, provides diagnostic information for an intraoperative cardiac event. It is essential to apply alpha-adrenergic agonists instead of beta-agonists for intraoperative hemodynamic support. The arrhythmias should be managed with beta-blockers or amiodarone. Adequate control of postoperative analgesia and nausea/vomiting is also important to suppress sympathetic activities.