46 Chapter 2 severe LVOT obstruction (n=2), and correction of an anomalous pulmonary vein (n=1). Of the total cohort, 7 patients underwent PCI and 4 CABG surgery. There were no patients who underwent a heart transplantation. Cardiac events in female patients with non-classical Fabry disease No MACEs were detected in women with non-classical FD. A total of 5 patients developed 8 non- major cardiac events (2 CA (1 tachycardia- bradycardia syndrome and 1 left/right bundle branch block for which a pacemaker was implanted, 1 AF, 3 moderate/ severe heart valve disease, 1 systolic dysfunction and 1 LVOT obstruction). All events in this group occurred from age 65 years onwards. MACE (excluding MI from the analysis) 13 men with classical FD, 4 men with non-classical FD and 8 women with classical FD experienced a MACE. None of the women with non-classical FD developed MACE. The event rate (after age 15 years) for MACE was 8.4 per 1000 patient-years for men with classical FD, versus 2.5 in women with classical FD, and 3.4 in men with non-classical FD. Median event- free survival was 60 years in men with classical FD, 75 years for women with classical FD. The median age of onset for MACE in men with non-classical FD was 67 years (p<0.001 for comparison men with classical FD versus the other three patient groups; p=0.11 for comparison men and women with classical FD). Supplemental table 1: Definitions of cardiac events 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
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