{"id":1515,"date":"2024-08-20T13:06:43","date_gmt":"2024-08-20T12:06:43","guid":{"rendered":"https:\/\/heliusstudy.nl\/?post_type=publicatie&#038;p=1515"},"modified":"2024-08-20T13:06:44","modified_gmt":"2024-08-20T12:06:44","slug":"ecg-changes-during-adult-life-in-fabry-disease-results-from-a-large-longitudinal-cohort-study","status":"publish","type":"publicatie","link":"https:\/\/heliusstudy.nl\/tr\/publicatie\/ecg-changes-during-adult-life-in-fabry-disease-results-from-a-large-longitudinal-cohort-study\/","title":{"rendered":"ECG Changes during Adult Life in Fabry Disease: Results from a Large Longitudinal Cohort Study"},"content":{"rendered":"\n<p><strong>Background:<\/strong>\u00a0Fabry disease (FD) is an X-linked, lysosomal storage disorder leading to severe cardiomyopathy in a significant proportion of patients. To identify ECG markers that reflect early cardiac involvement and disease progression, we conducted a long term retrospective study in a large cohort of FD patients.\u00a0<\/p>\n\n\n\n<p><strong>Methods:<\/strong>\u00a0A total of 1995 ECGs from 133 patients with classical FD (64% females, 80% treated with enzyme replacement therapy), spanning 20 years of follow-up, were compared to ECGs from 3893 apparently healthy individuals. Generalized linear mixed models were used to evaluate the effect of age, FD and sex on: P-wave duration, PR-interval, QRS-duration, QTc, Cornell index, spatial QRS-T angle and frontal QRS-axis. Regression slopes and absolute values for each parameter were compared between FD patients and control subjects.\u00a0<\/p>\n\n\n\n<p><strong>Results:<\/strong>\u00a0At a younger age (&lt;40 years), the Cornell index was higher and frontal QRS-axis more negative in FD patients compared to controls (<em>p<\/em>\u00a0&lt; 0.05). For the other ECG parameters, the rate of change, more than the absolute value, was greater in FD patients compared to controls (<em>p<\/em>\u00a0&lt; 0.05). From the fifth decade (men) or sixth (women) onwards, absolute values for P-wave duration, QRS-duration, QTc and spatial QRS-T angle were longer and higher in FD patients compared to control subjects.\u00a0<\/p>\n\n\n\n<p><strong>Conclusions:<\/strong>\u00a0ECG abnormalities indicative of FD are age and sex dependent. Tracking the rate of change in ECG parameters could be a good way to detect disease progression, guiding treatment initiation. Moreover, monitoring ECG changes in FD can be used to evaluate the effectiveness of treatment.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Background:\u00a0Fabry disease (FD) is an X-linked, lysosomal storage disorder leading to severe cardiomyopathy in a significant proportion of patients. To identify ECG markers that reflect early cardiac involvement and disease progression, we conducted a long term retrospective study in a large cohort of FD patients.\u00a0 Methods:\u00a0A total of 1995 ECGs from 133 patients with classical [&hellip;]<\/p>\n","protected":false},"featured_media":0,"template":"","meta":{"_acf_changed":true},"publications_year":[22],"thema":[42],"class_list":["post-1515","publicatie","type-publicatie","status-publish","hentry","publications_year-22","thema-cardiovascular-disease"],"acf":[],"_links":{"self":[{"href":"https:\/\/heliusstudy.nl\/tr\/wp-json\/wp\/v2\/publicatie\/1515","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/heliusstudy.nl\/tr\/wp-json\/wp\/v2\/publicatie"}],"about":[{"href":"https:\/\/heliusstudy.nl\/tr\/wp-json\/wp\/v2\/types\/publicatie"}],"wp:attachment":[{"href":"https:\/\/heliusstudy.nl\/tr\/wp-json\/wp\/v2\/media?parent=1515"}],"wp:term":[{"taxonomy":"publications_year","embeddable":true,"href":"https:\/\/heliusstudy.nl\/tr\/wp-json\/wp\/v2\/publications_year?post=1515"},{"taxonomy":"thema","embeddable":true,"href":"https:\/\/heliusstudy.nl\/tr\/wp-json\/wp\/v2\/thema?post=1515"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}