title: Clinical and genetic variables associated with acute arousal and nonarousal-related cardiac events among subjects with long QT syndrome.
abstract: In patients with the long QT syndrome (LQTS), the occurrence of cardiac events (syncope or cardiac arrest) is frequently associated with acute arousal caused by exercise, swimming, emotion, or noise. However, cardiac events may also occur during sleep or with ordinary daily activities. The purpose of this study was to determine whether there are differential clinical, electrocardiographic, and genetic features among LQTS patients who experienced cardiac events with and without acute arousal. We identified 1,325 patients with cardiac events from the International LQTS Registry. Based on the precipitating conditions of the first event, 427 patients were classified as arousal, 345 as nonarousal, and the remaining 553 were unknown (not classifiable). Gene linkage was known in 78 of the 772 patients with classifiable first events. The age at first cardiac event was significantly younger in the arousal than the nonarousal group (11.7 vs. 15.5 years, respectively; p<0.001). The arousal-type patients had a higher rate of subsequent cardiac events during follow-up after the index event than the nonarousal-type patients (p = 0.02). Arousal-related cardiac events occurred in 85% of LQT1, 67% of LQT2, and 33% of LQT3 patients (p = 0.008). This study provides evidence that the genotype is an important determinant of the LQTS phenotype in terms of arousal and nonarousal-related cardiac events.
authors.affiliation: Cardiology Unit, Long Island Jewish Hospital-North Shore Health System, Manhasset, New York, USA.
authors.forename: R H
authors.initials: RH
authors.lastname: Ali
authors.forename: W
authors.initials: W
authors.lastname: Zareba
authors.forename: A J
authors.initials: AJ
authors.lastname: Moss
authors.forename: P J
authors.initials: PJ
authors.lastname: Schwartz
authors.forename: J
authors.initials: J
authors.lastname: Benhorin
authors.forename: G M
authors.initials: GM
authors.lastname: Vincent
authors.forename: E H
authors.initials: EH
authors.lastname: Locati
authors.forename: S
authors.initials: S
authors.lastname: Priori
authors.forename: C
authors.initials: C
authors.lastname: Napolitano
authors.forename: J A
authors.initials: JA
authors.lastname: Towbin
authors.forename: W J
authors.initials: WJ
authors.lastname: Hall
authors.forename: J L
authors.initials: JL
authors.lastname: Robinson
authors.forename: M L
authors.initials: ML
authors.lastname: Andrews
authors.forename: L
authors.initials: L
authors.lastname: Zhang
authors.forename: K
authors.initials: K
authors.lastname: Timothy
authors.forename: A
authors.initials: A
authors.lastname: Medina
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D001143:Arousal / Q000235:genetics; D004562:Electrocardiography; D005260:Female; D008040:Genetic Linkage; D005838:Genotype; D006323:Heart Arrest / Q000209:etiology* / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D008133:Long QT Syndrome / Q000150:complications* / Q000235:genetics* / Q000503:physiopathology; D008297:Male; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D013312:Stress, Physiological / Q000150:complications; D015996:Survival Rate; D013575:Syncope / Q000209:etiology* / Q000235:genetics / Q000503:physiopathology