39 Cardiac outcomes in Fabry disease CVD, but this effect did not hold after correcting for multiple testing (table 3). Group comparisons showed significant higher risk in men with classical vs non-classical disease (HR: 22.2) and men versus women with classical disease (HR: 14.8) (table 4). HF was an important cause of CVD (40% of CVD in men with classical FD, 67% in women with classical FD, and 100% in men with nonclassical FD) (table 2, supplemental table 2). Heart failure hospitalization The event rate for HF hospitalization was 5.2 in men with classical FD, versus 1.9 in women with classical FD. In men with non-classical FD the event rate was 3.4. KM analysis showed a significant difference between the four subgroups (figure 4B, see supplemental figure 4 for CR analysis). Classical phenotype and male sex increased the risk of HF hospitalization, but this effect did not hold after correcting for multiple testing (table 3). Group comparisons showed significant higher risk in men with classical vs non-classical disease (HR: 15.5) and men versus women with classical disease (HR: 15.4) (table 4). The left ventricle ejection fraction prior or at the time of the event was known in 17 out of 18 patients, who were hospitalized because of HF. In 12/17 patients (71%), the ejection fraction was <50% (5/7 in men with classical FD, 3/6 in women with classical FD and 4/4 in men with non-classical FD). Sustained ventricular arrhythmias and implantable cardiac defibrillator device implantation The event rate of SVA was low (14 events in 9 patients in the total cohort) (figure 4C, see supplemental figure 5 for CR analysis, for between group comparison see table 3 and 4). The first SVA episode occurred at the time of an MI in 2 patients and in one patient at the time of HF hospitalization. Of the remaining 6 patients who experienced an SVA, 2 patients had an ejection fraction <35%, in 2 patients the first event occurred during hemodialysis, and in one patient an allergic reaction to clopidogrel was described as a possible trigger. In the last patient, no trigger could be identified. Of all patients who suffered SVA, 3 died as a result of the first episode (1 due to MI and 2 during hemodialysis), 1 patient died 3 years after the first SVA episode due to MI. The other 5 patients had an ICD implanted after the first event. In 3 patients, the ICD successfully aborted an episode of ventricular arrhythmia during follow-up. Of these 3 patients, 2 died of HF respectively 22 months and 6 years after ICD implantation. One patient was alive at last follow-up (ICD was 4.8 years in situ). In 2 patients, no shocks were administered by the ICD. Of these 2, 1 patient died 9 months after ICD implantation as a result of HF (no documented SVA), whereas in the other patient no shocks were documented at the time of a sudden cardiac arrest with pulseless electrical activity. He was resuscitated and survived the episode. In the 2
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