Heart rhythm : the official journal of the Heart Rhythm Society
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Risk stratification strategies employing sarcomere gene mutational analysis have proved imprecise in identifying high-risk patients with hypertrophic cardiomyopathy (HCM). Therefore, additional genetic risk markers that reliably determine which patients are predisposed to sudden death are needed. ⋯ These observations support the emerging hypothesis that double (or compound) mutations detected by genetic testing may confer a gene dosage effect in HCM, thereby predisposing patients to adverse disease progression. In 3 families, multiple sarcomere mutations were associated with a risk of sudden death, even in the absence of conventional risk factors.
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The commotio cordis literature has largely focused on events occurring in the United States. However, with enhanced public awareness, commotio cordis has been increasingly recognized internationally as a cause of cardiac arrest and sudden death due to blunt nonpenetrating chest blows. ⋯ Commotio cordis demonstrates a global occurrence, very similar demographically in the United States and internationally. However, the frequency with which chest blows from soccer balls caused commotio cordis events (particularly during sports played internationally) seems to contradict the prevailing notion that air-filled projectiles convey less risk for ventricular fibrillation than do those with solid cores (e.g., baseball or lacrosse balls).
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A growing body of evidence suggests that the arrhythmogenic substrate underlying Brugada syndrome (BrS) is located in the right ventricular outflow tract (RVOT), and electrophysiological abnormalities recently evidenced most commonly concur in conduction slowing. Also, imaging studies reported wall motion abnormalities of the RVOT in patients with BrS, with a various extent of RV remodeling. However, there are no data regarding a potential relationship between electrophysiological alterations and contraction abnormalities in BrS. ⋯ In patients with BrS, we found a relationship between RV contraction heterogeneity and ST-segment pattern, providing evidence of a functional modulation of the arrhythmogenic substrate.
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The diagnosis of subtle structural heart disease in competitive athletes with ventricular arrhythmias (VAs) and an apparently normal heart is challenging. Three-dimensional electroanatomic mapping (EAM) has been demonstrated to reliably identify low-voltage areas that correspond to different cardiomyopathic substrates. ⋯ Electroanatomical substrate mapping may help diagnose concealed myocardial diseases in competitive athletes presenting with recent-onset VAs and an apparently normal heart. Further studies are warranted to assess the prognostic implications of such subtle myocardial abnormalities.