Article
Natural History and Risk Stratification in Andersen-Tawil Syndrome Type 1.
Journal of the American College of Cardiology - 21 Apr 2020
Mazzanti Andrea, Guz Dmitri, Trancuccio Alessandro, Pagan Eleonora, Kukavica Deni, Chargeishvili Tekla, Olivetti Natalia, Biernacka Elżbieta Katarzyna, Sacilotto Luciana, Sarquella-Brugada Georgia, Campuzano Oscar, Nof Eyal, Anastasakis Aristides, Sansone Valeria A, Jimenez-Jaimez Juan, Cruz Fernando, Sánchez-Quiñones Jessica, Hernandez-Afonso Julio, Fuentes Maria Eugenia, Średniawa Beata, Garoufi Anastasia, Andršová Irena, Izquierdo Maite, Marinov Rumen, Danon Asaf, Expósito-García Victor, Garcia-Fernandez Amaya, Muñoz-Esparza Carmen, Ortíz Martín, Zienciuk-Krajka Agnieszka, Tavazzani Elisa, Monteforte Nicola, Bloise Raffaella, Marino Maira, Memmi Mirella, Napolitano Carlo, Zorio Esther, Monserrat Lorenzo, Bagnardi Vincenzo, Priori Silvia G
Abstract excerpt
BACKGROUND: Andersen-Tawil Syndrome type 1 (ATS1) is a rare arrhythmogenic disorder, caused by loss-of-function mutations in the KCNJ2 gene. We present here the largest cohort of patients with ATS1 with outcome data reported. OBJECTIVES: 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...
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