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Published on: December 11, 2017
Inherited heart rhythm disease: negotiating the minefield for the practicing cardiologist
Andrew D Krahn1, Shubhayan Sanatani, Martin J Gardner
1Division of Cardiology, University of British Columbia, Vancouver, British Columbia, Canada. akrahn@mail.ubc.ca
Insights
Inherited heart rhythm disorders, including channelopathies and cardiomyopathies, pose diagnostic and management challenges. This review offers guidance for clinicians and highlights resources for improved patient care and family screening.
Area of Science:
- Cardiology
- Genetics
- Clinical Medicine
Background:
- Inherited heart rhythm disorders are uncommon yet challenging for clinicians.
- Key electrical disorders include long QT syndrome, Brugada syndrome, and catecholaminergic ventricular tachycardia.
- Inherited cardiomyopathies like hypertrophic and arrhythmogenic right ventricular cardiomyopathy also cause arrhythmias and sudden death.
Purpose of the Study:
- To review diagnostic considerations for inherited heart rhythm disorders.
- To provide guidance for the day-to-day management of these conditions.
- To highlight accessible resources for healthcare professionals and patients.
Main Methods:
- Review of key historic and diagnostic considerations.
- Discussion of management strategies and accessible resources.
- Emphasis on disease-specific treatments and family screening.
Main Results:
- Inherited heart rhythm disorders present diagnostic and management difficulties.
- Accessible resources and expert clinics can aid healthcare providers.
- Common measures can assist providers and reduce patient risk.
Conclusions:
- Effective management of inherited heart rhythm disorders requires careful diagnosis and tailored treatment.
- Utilizing expert networks and available resources improves patient outcomes.
- Family screening is crucial for identifying at-risk individuals.
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.
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