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

  • Kristina H. HaugaaEmail author
  • Ida S. Leren
Women and Heart Disease (C. Linde, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Women and Heart Disease


Purpose of Review

With 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 Findings

Despite 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.


Important 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.


Long QT syndrome Brugada syndrome Arrhythmogenic cardiomyopathy Hypertrophic cardiomyopathy Gender differences Sex differences 


Compliance with Ethical Standards

Conflict of Interest

Ida S. Leren and Kristina H. Haugaa declare no conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Cardiology and Center for Cardiological InnovationOslo University Hospital, RikshospitaletOsloNorway
  2. 2.University of OsloOsloNorway

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