Prevalence, Clinical Presentation, and Management of Channelopathies and Cardiomyopathies, Long QT Syndrome, Brugada Syndrome, Arrhythmogenic Cardiomyopathy, and Hypertrophic Cardiomyopathy

Purpose of ReviewWith this paper, we aim to summarize the knowledge on gender differences in the most common inheritable channelopathies and cardiomyopathies, focusing on aspects that are of clinical importance for patient management and follow-up.Recent FindingsDespite autosomal dominant inheritance patterns in most of the inheritable cardiac channelopathies and cardiomyopathies, there is increasing awareness that there are important gender differences in disease penetrance and severity, affecting prevalence, clinical presentation, and patient management.SummaryImportant gender differences are present in Long QT syndrome, Brugada syndrome, arrhythmogenic cardiomyopathy, and hypertrophic cardiomyopathy. In LQTS, genotype-specific differences are important, and female LQT2 patients have higher arrhythmic risk compared with male. In the remaining inheritable channelopathies and cardiomyopathies discussed in this review, male patients are more likely to have penetrant disease and experience arrhythmic events. Mechanistic explanations for the observed gender differences are sparse, but in channelopathies, hormonal effects are thought to be important. Although treatment strategies in inheritable channelopathies and cardiomyopathies are similar in men and women with the notable exception of women with LQT2, the differences between the sexes are important to be aware of in patient management.