96 Chapter 3 Supplemental table 3: Definitions of cardiac events [1] Events Definition Major adverse cardiovascular events (MACE): composite of cardiovascular death, heart failure hospitalization, sustained ventricular arrhythmias (SVA) and myocardial infarction 1. Cardiovascular death Death as a result of one of the following diseases/ syndromes: - Acute coronary syndrome - Sudden cardiac death (SCD) - Hypertensive crise - Ischemic or hemorrhagic stroke - Cardiomyopathy - Other cardiovascular cause such as: pulmonary embolism, peripheral vascular disease 2. Heart failure hospitalization Hospital admission (at least one night) with the following clinical manifestations of heart failure: dyspnea, reduced exercise tolerance, fluid retention in peripheral and/ or splanchnic vessels, seen as peripheral edema 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 1. El Sayed, M., et al., Influence of sex and phenotype on cardiac outcomes in patients with Fabry disease. Heart, 2021. Supplemental table 4: GLA mutations frequencies GLA mutation at DNA level Frequency in FD- men (N) Frequency in FD- women (N) c.1025G>A 8 13 c.1040dupT 0 2 c.1074_1075del 1 3 c.1118G>A 1 2 c.1124_1176del 2 0 c.1156C>T 2 1 c.1246C>T 1 1 c.136C>T 0 1 c.157_160delAACC 2 1 c.215T>G 1 0 c.269G>A 0 1 c.334C>T 1 1
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