Inherited cardiomyopathies.

Editor—It was interesting to read Staikou and colleagues' 1 excellent review of Perioperative management of hereditary arrhythmogenic syndromes. This group of disorders seems to have previously been under represented in mainstream anaesthetic literature. The collective incidence of the group of disorders that are described means that all consultant anaesthetists will have anaesthetized a number of patients who will subsequently present with one of these disorders. The fact that presentation is often with sudden death raises obvious questions as to whether a number of these deaths could be prevented by earlier identification. The majority of those presenting for anaesthesia with such an underlying condition are likely to arrive with no confirmed diagnosis. Diagnosis of these conditions may be difficult as a large number of genotypes can result in different ion channel or other structural abnormalities that result in similar phenotypic variations, which may be characterized under one diagnosis. Variable penetrance and the fact that presentation of these congenital abnormalities is often not before adolescence, or later in life, makes the identification of familial patterns difficult. Traditional teaching has been that ventricular extrasys-toles are not uncommon in middle age and in patients with no ischaemic heart disease or obvious cardiomyopathy are usually of no clinical significance. Similarly, occasional short runs of bigemeni noted on ECG monitors are often assumed to be benign if they resolve spontaneously and do not result in any detectable short-term consequences. Anaesthetists watching ECGs for extended periods of time during otherwise uneventful cases are ideally placed to identify and highlight certain individuals for investigation. Is it now time for anaesthetists to have a higher index of suspicion when they observe frequent signs of ventricular ectopy that do not result in short-term detrimental effects? A post-operative discussion to elicit any family history of unexplained or early cardiac death, 24 h ECG monitoring, and an echocardiogram would seem to be appropriate steps to take in any patient showing abnormal ectopy. The challenge now, in an era of finite resources, must be to define the level at which to act. 1 and his constructive comments regarding the role of anaes-thesiologists in early diagnosis of hereditary arrhythmogenic syndromes in suspicious cases. We agree that arrhythmogenic syndromes are underdiag-nosed, since they may be asymptomatic for a long time. Thus, several anaesthesiologists have probably provided, or will provide at some time, anaesthesia to an undiagnosed patient. Since most of the times the postoperative outcome …