Ventricular Tachycardia in Fabry Disease Detected in a 50-Year-Old Woman during 14-Day Continuous Cardiac Monitoring
Fabry disease is an X-linked lysosomal storage disorder. Female carriers were long thought to be asymptomatic; however, research has revealed the opposite. Cardiac conditions are the chief causes of death in women with Fabry disease. Although ventricular tachycardia has been reported in male patient...
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| Veröffentlicht in: | Texas Heart Institute journal 2016-12, Vol.43 (6), p.531-533 |
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| creator | Silva-Gburek, Jaime Rochford, Laura Hopkin, Robert Jefferies, John L |
| description | Fabry disease is an X-linked lysosomal storage disorder. Female carriers were long thought to be asymptomatic; however, research has revealed the opposite. Cardiac conditions are the chief causes of death in women with Fabry disease. Although ventricular tachycardia has been reported in male patients with Fabry disease, it is not thought to be a frequent finding in females. We describe the case of a 50-year-old woman in whom we used 14-day continuous electrocardiographic monitoring to identify nonsustained ventricular tachycardia, after electrocardiograms and 24-hour Holter monitoring failed to detect the arrhythmia. A permanent implantable cardioverter-defibrillator relieved the patient's symptoms. We discuss why this case supports the need for more extensive electrophysiologic evaluation in women who have Fabry disease. |
| doi_str_mv | 10.14503/THIJ-15-5572 |
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Female carriers were long thought to be asymptomatic; however, research has revealed the opposite. Cardiac conditions are the chief causes of death in women with Fabry disease. Although ventricular tachycardia has been reported in male patients with Fabry disease, it is not thought to be a frequent finding in females. We describe the case of a 50-year-old woman in whom we used 14-day continuous electrocardiographic monitoring to identify nonsustained ventricular tachycardia, after electrocardiograms and 24-hour Holter monitoring failed to detect the arrhythmia. A permanent implantable cardioverter-defibrillator relieved the patient's symptoms. 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Female carriers were long thought to be asymptomatic; however, research has revealed the opposite. Cardiac conditions are the chief causes of death in women with Fabry disease. Although ventricular tachycardia has been reported in male patients with Fabry disease, it is not thought to be a frequent finding in females. We describe the case of a 50-year-old woman in whom we used 14-day continuous electrocardiographic monitoring to identify nonsustained ventricular tachycardia, after electrocardiograms and 24-hour Holter monitoring failed to detect the arrhythmia. A permanent implantable cardioverter-defibrillator relieved the patient's symptoms. We discuss why this case supports the need for more extensive electrophysiologic evaluation in women who have Fabry disease.</description><subject>alpha-Galactosidase - therapeutic use</subject><subject>Case Reports</subject><subject>Defibrillators, Implantable</subject><subject>Electric Countershock - instrumentation</subject><subject>Electrocardiography, Ambulatory - methods</subject><subject>Electrophysiologic Techniques, Cardiac</subject><subject>Enzyme Replacement Therapy</subject><subject>Fabry Disease - complications</subject><subject>Fabry Disease - diagnosis</subject><subject>Fabry Disease - drug therapy</subject><subject>Fatigue - etiology</subject><subject>Female</subject><subject>Humans</subject><subject>Middle Aged</subject><subject>Predictive Value of Tests</subject><subject>Syncope - etiology</subject><subject>Tachycardia, Ventricular - diagnosis</subject><subject>Tachycardia, Ventricular - etiology</subject><subject>Tachycardia, Ventricular - physiopathology</subject><subject>Tachycardia, Ventricular - therapy</subject><subject>Time Factors</subject><subject>Treatment Outcome</subject><issn>0730-2347</issn><issn>1526-6702</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpVkb1vFDEUxC0UlBwhJS1ymcbwbK_Xu00kdJcvFJTmAFFZzx9JHO3Zwd6NdP89t0mIoHrF_DTzRkPIBw6feKNAfl5fXH5lXDGltHhDFlyJlrUaxB5ZgJbAhGz0AXlX6z0ASMHFPjkQHQfodbsg44-QxhLdNGCha3R3W4fFR6Qx0TO0ZUtXsQasga7CGNwY_KwgVcB-BSzsevD0Z95gon4qMd1S3rAVbukypzGmKU-VLp8MHf2WUxzzDL0nb29wqOHo5R6S72en6-UFu7o-v1x-uWJOdnpkwUvfgLZS9Qp9Y0E0nfUQILTKKt31fQDROdtyyyUoFNALjqi5s0Jp1clDcvLs-zDZTfBuroqDeShxg2VrMkbzv5LinbnNj0Zx3fNW7AyOXwxK_j2FOppNrC4MA6awq2Z41_JdkupmlD2jruRaS7h5jeFgnpYy81KGKzMvteM__vvbK_13GvkH26qOwQ</recordid><startdate>201612</startdate><enddate>201612</enddate><creator>Silva-Gburek, Jaime</creator><creator>Rochford, Laura</creator><creator>Hopkin, Robert</creator><creator>Jefferies, John L</creator><general>Texas Heart® Institute, Houston</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>201612</creationdate><title>Ventricular Tachycardia in Fabry Disease Detected in a 50-Year-Old Woman during 14-Day Continuous Cardiac Monitoring</title><author>Silva-Gburek, Jaime ; Rochford, Laura ; Hopkin, Robert ; Jefferies, John L</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c387t-ed3d407b3595ad4b0248bd0e0e65b57899e028cb61b1305a20921aa71cb257583</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2016</creationdate><topic>alpha-Galactosidase - therapeutic use</topic><topic>Case Reports</topic><topic>Defibrillators, Implantable</topic><topic>Electric Countershock - instrumentation</topic><topic>Electrocardiography, Ambulatory - methods</topic><topic>Electrophysiologic Techniques, Cardiac</topic><topic>Enzyme Replacement Therapy</topic><topic>Fabry Disease - complications</topic><topic>Fabry Disease - diagnosis</topic><topic>Fabry Disease - drug therapy</topic><topic>Fatigue - etiology</topic><topic>Female</topic><topic>Humans</topic><topic>Middle Aged</topic><topic>Predictive Value of Tests</topic><topic>Syncope - etiology</topic><topic>Tachycardia, Ventricular - diagnosis</topic><topic>Tachycardia, Ventricular - etiology</topic><topic>Tachycardia, Ventricular - physiopathology</topic><topic>Tachycardia, Ventricular - therapy</topic><topic>Time Factors</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Silva-Gburek, Jaime</creatorcontrib><creatorcontrib>Rochford, Laura</creatorcontrib><creatorcontrib>Hopkin, Robert</creatorcontrib><creatorcontrib>Jefferies, John L</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Texas Heart Institute journal</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Silva-Gburek, Jaime</au><au>Rochford, Laura</au><au>Hopkin, Robert</au><au>Jefferies, John L</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Ventricular Tachycardia in Fabry Disease Detected in a 50-Year-Old Woman during 14-Day Continuous Cardiac Monitoring</atitle><jtitle>Texas Heart Institute journal</jtitle><addtitle>Tex Heart Inst J</addtitle><date>2016-12</date><risdate>2016</risdate><volume>43</volume><issue>6</issue><spage>531</spage><epage>533</epage><pages>531-533</pages><issn>0730-2347</issn><eissn>1526-6702</eissn><abstract>Fabry disease is an X-linked lysosomal storage disorder. 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| ispartof | Texas Heart Institute journal, 2016-12, Vol.43 (6), p.531-533 |
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| source | MEDLINE; PubMed Central; Alma/SFX Local Collection |
| subjects | alpha-Galactosidase - therapeutic use Case Reports Defibrillators, Implantable Electric Countershock - instrumentation Electrocardiography, Ambulatory - methods Electrophysiologic Techniques, Cardiac Enzyme Replacement Therapy Fabry Disease - complications Fabry Disease - diagnosis Fabry Disease - drug therapy Fatigue - etiology Female Humans Middle Aged Predictive Value of Tests Syncope - etiology Tachycardia, Ventricular - diagnosis Tachycardia, Ventricular - etiology Tachycardia, Ventricular - physiopathology Tachycardia, Ventricular - therapy Time Factors Treatment Outcome |
| title | Ventricular Tachycardia in Fabry Disease Detected in a 50-Year-Old Woman during 14-Day Continuous Cardiac Monitoring |
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