47 Cardiac outcomes in Fabry disease Supplemental table 1: Definitions of cardiac events (continued) Events Definition 3. Sustained ventricular arrhythmia (SVA) composite of sudden cardiac death (SCD), sudden cardiac arrest (SCA), sustained ventricular tachycardia (VT) including appropriate ICD shock, and ventricular fibrillation (VF) 4. Myocardial infarction* Acute myocardial injury with clinical evidence of acute myocardial ischemia. Definition according to the Fourth Universal Definition of Myocardial Infarction Sustained ventricular arrhythmias (SVA): composite of sudden cardiac death (SCD), sudden cardiac arrest (SCA), sustained ventricular tachycardia (VT) including appropriate ICD shock, and ventricular fibrillation (VF) 5. Sudden cardiac death* Sudden cessation of cardiac activity, resulting in hemodynamic collapse and death. Often typically due to sustained ventricular arrhythmias (VT, VF). This occurs either : - ≤ 1 hour after observed cardiac symptoms/ abnormalities or - If the patient is found dead, he or she was seen alive in the previous 24 hours without cardiac symptoms. Trauma, overdose, drowning and suicide exclude SCD 6. Sudden cardiac arrest* Sudden cessation of cardiac activity so that the person becomes unresponsive, with no normal breathing and no signs of circulation 7. Sustained ventricular tachycardia* Ventricular tachycardia lasting for >30 seconds or ended <30 seconds by a defibrillator 8. Ventricular fibrillation* Inconsistent depolarization of the ventricles with AV- dissociation resulting in mechanical cardiac arrest (resuscitationand/or defibrillation often necessary) Conduction abnormalities (CA): composite of second-degree atrioventricular (AV) block Mobitz II, third-degree AV block, sinusarrest (SA) and pacemaker (PM) or implantable cardiac defibrillator device (ICD) implantation for conduction abnormalities 9. Second-degree AV block, Mobitz II Intermittent nonconducted P waves not preceded by PR prolongation and not followed by PR shortening. 10. Third-degree AV block Interruption of impulse transmission from the atria to the ventricles 2
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