Natural history and risk stratification in Andersen-Tawil Sy
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Andersen-Tawil Syndrome type 1 (ATS1) is a rare arrhythmogenic disorder, caused by loss-of-function mutations in the KCNJ2 gene. This report presents the largest cohort of patients with ATS1 with outcome data reported.

This study sought to define the risk of life-threatening arrhythmic events (LAE), identify predictors of such events, and define the efficacy of antiarrhythmic therapy in patients with ATS1. Clinical and genetic data from consecutive patients with ATS1 from 23 centers were entered in a database implemented at ICS Maugeri in Pavia, Italy, and pooled for analysis.

Results: It enrolled 118 patients with ATS1 from 57 families (age 23 ± 17 years at enrollment). Over a median follow-up of 6.2 years, 17 patients experienced a first LAE, with a cumulative probability of 7.9% at 5 years. An increased risk of LAE was associated with a history of syncope, with the documentation of sustained ventricular tachycardia and with the administration of amiodarone. The rate of LAE without therapy was not reduced by beta-blockers alone, or in combination with Class Ic antiarrhythmic drugs.

Conclusively, this data demonstrates that the clinical course of patients with ATS1 is characterized by a high rate of LAE. A history of unexplained syncope or of documented sustained ventricular tachycardia is associated with a higher risk of LAE. Amiodarone is proarrhythmic and should be avoided in patients with ATS1.

Source: http://www.onlinejacc.org/content/75/15/1772?rss=1
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