title: Inherited heart rhythm disease: negotiating the minefield for the practicing cardiologist.
abstract: Inherited heart rhythm disorders are uncommon conditions that have emerged as a challenge to recognize and treat for the practicing clinician. The common electrical forms are long QT and Brugada syndrome, catecholaminergic ventricular tachycardia, and early repolarization syndrome. Inherited cardiomyopathies, including hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy, and left ventricular noncompaction can also cause serious cardiac rhythm disturbances and sudden death. We review the key historic and diagnostic considerations to provide guidance for day-to-day management, and the resources accessible to health care professionals and patients including a Canadian network of expert clinics. This allows for the initiation of disease-specific treatments and enables family screening. These heterogenous conditions can be challenging to diagnose with equally difficult management decisions. However, some common measures can be applied that will assist health care providers and reduce risk for patients. Condition-specific treatment strategies that are deployed in regional clinics are discussed, including the role of the referring health care team.
authors.affiliation: Division of Cardiology, University of British Columbia, Vancouver, British Columbia, Canada. akrahn@mail.ubc.ca
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Krahn
authors.forename: Shubhayan
authors.initials: S
authors.lastname: Sanatani
authors.forename: Martin J
authors.initials: MJ
authors.lastname: Gardner
authors.forename: Laura
authors.initials: L
authors.lastname: Arbour
mesh_terms: D001145:Arrhythmias, Cardiac / Q000235:genetics*; D002309:Cardiology / Q000592:standards*; D020022:Genetic Predisposition to Disease; D006801:Humans; D017008:Negotiating; D010348:Patient Care Team / Q000592:standards*; D017410:Practice Guidelines as Topic