Natural History and Risk Stratification in Andersen-Tawil Syndrome Type 1.
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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
Аннотация
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 patients with ATS1.
METHODS: 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: We enrolled 118 patients with ATS1 from 57 families (age 23 ± 17 years at enrollment). Over a median follow-up of 6.2 years (interquartile range: 2.7 to 16.5 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 (hazard ratio [HR]: 4.54; p = 0.02), with the documentation of sustained ventricular tachycardia (HR 9.34; p = 0.001) and with the administration of amiodarone (HR: 268; p < 0.001). The rate of LAE without therapy (1.24 per 100 person-years [py]) was not reduced by beta-blockers alone (1.37 per 100 py; p = 1.00), or in combination with Class Ic antiarrhythmic drugs (1.46 per 100 py, p = 1.00).
CONCLUSIONS: Our data demonstrate 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.