Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry Disease
Fabry disease (FD) is an X-linked disorder with α-galactosidase A deficiency. Males (>30 years) and females (>40 years) often present with cardiac manifestations, predominantly left ventricular hypertrophy (LVH). The aim of this study was to evaluate electrocardiographic (ECG) characteristics...
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MDPI AG
2022-01-01
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| Series: | Journal of Cardiovascular Development and Disease |
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| Online Access: | https://www.mdpi.com/2308-3425/9/1/11 |
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| author | Matthew Zada Queenie Lo Siddharth J. Trivedi Mehmet Harapoz Anita C. Boyd Kerry Devine Norman Sadick Michel C. Tchan Liza Thomas |
| author_facet | Matthew Zada Queenie Lo Siddharth J. Trivedi Mehmet Harapoz Anita C. Boyd Kerry Devine Norman Sadick Michel C. Tchan Liza Thomas |
| author_sort | Matthew Zada |
| collection | DOAJ |
| description | Fabry disease (FD) is an X-linked disorder with α-galactosidase A deficiency. Males (>30 years) and females (>40 years) often present with cardiac manifestations, predominantly left ventricular hypertrophy (LVH). The aim of this study was to evaluate electrocardiographic (ECG) characteristics within FD patients to identify gender related differences, and to additionally explore the association of ECG parameters with structural and functional alterations on transthoracic echocardiography (TTE). Retrospective cross-sectional analysis of 45 FD patients with contemporaneous ECG and TTE was performed and compared to age and gender matched healthy controls. FD patients demonstrated alterations in several ECG parameters particularly in males, including prolonged P-wave duration (91 vs. 81 ms, <i>p</i> = 0.022), prolonged QRS duration (96 vs. 84 ms, <i>p</i> < 0.001), increased R-wave amplitude in lead I (8.1 vs. 5.7 mV, <i>p</i> = 0.047), increased Sokolow–Lyon index (25 vs. 19 mV, <i>p</i> = 0.002) and were more likely to meet LVH criteria (31% vs. 7%, <i>p</i> = 0.006). FD patients with impaired basal longitudinal strain (LS) on TTE were more likely to meet LVH criteria (41% vs. 0%, <i>p</i> = 0.018). Those with more advanced FD (increased LV wall thickness on TTE) were more likely to meet LVH criteria but additionally demonstrated prolonged ventricular depolarization (QRS duration 101 vs. 88 ms, <i>p</i> = 0.044). Therefore, alterations on ECG demonstrating delayed atrial activation, delayed ventricular depolarization and evidence of LVH were more often seen in male FD patients. Impaired basal LS, a TTE marker of early cardiac involvement, correlated with ECG abnormalities. Increased LV wall thickness on TTE, a marker of more advanced FD, was associated with more severe ECG abnormalities. |
| first_indexed | 2024-03-10T01:15:07Z |
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| institution | Directory Open Access Journal |
| issn | 2308-3425 |
| language | English |
| last_indexed | 2024-03-10T01:15:07Z |
| publishDate | 2022-01-01 |
| publisher | MDPI AG |
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| series | Journal of Cardiovascular Development and Disease |
| spelling | doaj.art-5bf1ef9a61cf4ef1a4c066da5cb3f5312023-11-23T14:11:03ZengMDPI AGJournal of Cardiovascular Development and Disease2308-34252022-01-01911110.3390/jcdd9010011Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry DiseaseMatthew Zada0Queenie Lo1Siddharth J. Trivedi2Mehmet Harapoz3Anita C. Boyd4Kerry Devine5Norman Sadick6Michel C. Tchan7Liza Thomas8Westmead Clinical School, Westmead Hospital, University of Sydney, Sydney 2145, AustraliaSouth Western Sydney Clinical School, Liverpool Hospital, University of New South Wales, Sydney 1466, AustraliaWestmead Clinical School, Westmead Hospital, University of Sydney, Sydney 2145, AustraliaWestmead Clinical School, Westmead Hospital, University of Sydney, Sydney 2145, AustraliaWestmead Private Cardiology, Sydney 2145, AustraliaGenetic Medicine, Westmead Hospital, Sydney 2145, AustraliaWestmead Clinical School, Westmead Hospital, University of Sydney, Sydney 2145, AustraliaGenetic Medicine, Westmead Hospital, Sydney 2145, AustraliaWestmead Clinical School, Westmead Hospital, University of Sydney, Sydney 2145, AustraliaFabry disease (FD) is an X-linked disorder with α-galactosidase A deficiency. Males (>30 years) and females (>40 years) often present with cardiac manifestations, predominantly left ventricular hypertrophy (LVH). The aim of this study was to evaluate electrocardiographic (ECG) characteristics within FD patients to identify gender related differences, and to additionally explore the association of ECG parameters with structural and functional alterations on transthoracic echocardiography (TTE). Retrospective cross-sectional analysis of 45 FD patients with contemporaneous ECG and TTE was performed and compared to age and gender matched healthy controls. FD patients demonstrated alterations in several ECG parameters particularly in males, including prolonged P-wave duration (91 vs. 81 ms, <i>p</i> = 0.022), prolonged QRS duration (96 vs. 84 ms, <i>p</i> < 0.001), increased R-wave amplitude in lead I (8.1 vs. 5.7 mV, <i>p</i> = 0.047), increased Sokolow–Lyon index (25 vs. 19 mV, <i>p</i> = 0.002) and were more likely to meet LVH criteria (31% vs. 7%, <i>p</i> = 0.006). FD patients with impaired basal longitudinal strain (LS) on TTE were more likely to meet LVH criteria (41% vs. 0%, <i>p</i> = 0.018). Those with more advanced FD (increased LV wall thickness on TTE) were more likely to meet LVH criteria but additionally demonstrated prolonged ventricular depolarization (QRS duration 101 vs. 88 ms, <i>p</i> = 0.044). Therefore, alterations on ECG demonstrating delayed atrial activation, delayed ventricular depolarization and evidence of LVH were more often seen in male FD patients. Impaired basal LS, a TTE marker of early cardiac involvement, correlated with ECG abnormalities. Increased LV wall thickness on TTE, a marker of more advanced FD, was associated with more severe ECG abnormalities.https://www.mdpi.com/2308-3425/9/1/11Fabry diseaseelectrocardiogramtransthoracic echocardiographyLV hypertrophyglobal longitudinal strainbasal longitudinal strain |
| spellingShingle | Matthew Zada Queenie Lo Siddharth J. Trivedi Mehmet Harapoz Anita C. Boyd Kerry Devine Norman Sadick Michel C. Tchan Liza Thomas Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry Disease Journal of Cardiovascular Development and Disease Fabry disease electrocardiogram transthoracic echocardiography LV hypertrophy global longitudinal strain basal longitudinal strain |
| title | Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry Disease |
| title_full | Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry Disease |
| title_fullStr | Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry Disease |
| title_full_unstemmed | Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry Disease |
| title_short | Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry Disease |
| title_sort | electrocardiographic characteristics and their correlation with echocardiographic alterations in fabry disease |
| topic | Fabry disease electrocardiogram transthoracic echocardiography LV hypertrophy global longitudinal strain basal longitudinal strain |
| url | https://www.mdpi.com/2308-3425/9/1/11 |
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