Executive Summary: HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes

• Published in: Journal of arrhythmia 2014-02, Vol.30 (1), p.29-47
• Main Authors: Priori, Silvia G., MD, PhD, Wilde, Arthur A., MD, PhD, Horie, Minoru, MD, PhD, Cho, Yongkeun, MD, PhD, Behr, Elijah R., MA, MBBS, MD, FRCP, Berul, Charles, MD, FHRS, CCDS, Blom, Nico, MD, PhD, Brugada, Josep, MD, PhD, Chiang, Chern-En, MD, PhD, Huikuri, Heikki, MD, Kannankeril, Prince, MD, Krahn, Andrew, MD, FHRS, Leenhardt, Antoine, MD, Moss, Arthur, MD, Schwartz, Peter J., MD, Shimizu, Wataru, MD, PhD, Tomaselli, Gordon, MD, FHRS, Tracy, Cynthia, MD
• Format: Article
• Language: English
• Subjects: Associations, institutions, etc; Asymptomatic; Beta blockers; Cardiac arrest; Cardiac arrhythmia; Cardiac patients; Cardiovascular; Care and treatment; Contraindications; Electrocardiography; Electrolytes; Genetic disorders; Genetic testing; Heart; Heart beat; Mutation; Patients; Societies; Writing
• ISSN: 1880-4276; 1883-2148
• DOI: 10.1016/j.joa.2013.08.001

Guidance for the evaluation of patients with idiopathic ventricular fibrillation, sudden arrhythmic death syndrome, and sudden unexplained death in infancy, which includes genetic testing, are provided as these topics were not covered in the previous consensus statement. Documents produced by other medical societies have acknowledged the need to define the criteria used to rank the strength of recommendation for genetic diseases. 2 The most obvious difference encountered for inherited diseases is that randomized and/or blinded studies do not exist in this field. [...]most of the available data derive from registries that have followed patients and recorded outcome information. The following lifestyle changes are recommended in all patients with a diagnosis of LQTS: a. Avoidance of QT-prolonging drugs (www.qtdrugs.org) b. Identification and correction of electrolyte abnormalities that may occur during diarrhea, vomiting, metabolic conditions, or imbalanced diets for weight loss. 2. A scoring system has been established, which takes into account the age of the patient, medical and family history, symptoms, and QTc and provides a probability of the diagnosis of LQTS. 7,8 Approximately 20%–25% of the patients with LQTS confirmed by the presence of an LQTS gene mutation may have a normal range QTc. 9,10 Risk stratification