Purpose of review: The identification of the first genes for the long QT syndrome (LQTS) in 1995 has spurred awareness among clinical cardiologists of cardiac ion channel diseases, resulting in more frequent diagnoses. Hence, there is a growing need to know more about the management of these complex and often lethal diseases. The present review addresses these practical issues.
Recent findings: Besides information on the genetic bases and prevalence, this review focuses primarily on the current state of therapy and management. This ranges from traditional and valid therapies to newer approaches still requiring validation to recent advances in gene-specific management. The significant problems generated by the growing use of the implantable cardioverter defibrillator (ICD) in children with the attendant impact on their quality of life are considered and attention is given to effective therapies which lessen the need to resort to an often life-saving therapy which in children, however, is burdened by major negative side-effects.
Summary: The findings described will allow practicing cardiologists to face their young patients affected by cardiac ion channel diseases with greater confidence to reach the best treatment and to have a better understanding of the special medical problems posed by these intriguing disorders.