Congenital Short QT Syndrome
Long QT intervals in the ECG have long been associated with sudden cardiac death. The congenital long QT syndrome was first described in individuals with structurally normal hearts in 1957.1 Little was known about the significance of a short QT interval. In 1993, after analyzing 6693 consecutive H...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Elsevier
2004-04-01
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| Series: | Indian Pacing and Electrophysiology Journal |
| Subjects: | |
| Online Access: | http://www.ipej.org/0402/antzelevitch.htm |
| _version_ | 1818155090583748608 |
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| author | Charles Antzelevitch Johnson Francis |
| author_facet | Charles Antzelevitch Johnson Francis |
| author_sort | Charles Antzelevitch |
| collection | DOAJ |
| description | Long QT intervals in the ECG have long been associated with sudden cardiac death. The congenital long QT syndrome was first described in individuals with structurally normal hearts in 1957.1 Little was known about the significance of a short QT interval. In 1993, after analyzing 6693 consecutive Holter recordings Algra et al concluded that an increased risk of sudden death was present not only in patients with long QT interval, but also in patients with short QT interval (<400 ms).2 Because this was a retrospective analysis, further evaluation of the data was not possible. It was not until 2000 that a short-QT syndrome (SQTS) was proposed as a new inherited clinical syndrome by Gussak et al.3 The initial report was of two siblings and their mother all of whom displayed persistently short QT interval. The youngest was a 17 year old female presenting with several episodes of paroxysmal atrial fibrillation requiring electrical cardioversion.3 Her QT interval measured 280 msec at a heart rate of 69. Her 21 year old brother displayed a QT interval of 272 msec at a heart rate of 58, whereas the 51 year old mother showed a QT of 260 msec at a heart rate of 74. The authors also noted similar ECG findings in another unrelated 37 year old patient associated with sudden cardiac death. |
| first_indexed | 2024-12-11T14:36:53Z |
| format | Article |
| id | doaj.art-1785315a6b1948d69df29a37113bfee2 |
| institution | Directory Open Access Journal |
| issn | 0972-6292 |
| language | English |
| last_indexed | 2024-12-11T14:36:53Z |
| publishDate | 2004-04-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Indian Pacing and Electrophysiology Journal |
| spelling | doaj.art-1785315a6b1948d69df29a37113bfee22022-12-22T01:02:08ZengElsevierIndian Pacing and Electrophysiology Journal0972-62922004-04-01424649Congenital Short QT SyndromeCharles AntzelevitchJohnson FrancisLong QT intervals in the ECG have long been associated with sudden cardiac death. The congenital long QT syndrome was first described in individuals with structurally normal hearts in 1957.1 Little was known about the significance of a short QT interval. In 1993, after analyzing 6693 consecutive Holter recordings Algra et al concluded that an increased risk of sudden death was present not only in patients with long QT interval, but also in patients with short QT interval (<400 ms).2 Because this was a retrospective analysis, further evaluation of the data was not possible. It was not until 2000 that a short-QT syndrome (SQTS) was proposed as a new inherited clinical syndrome by Gussak et al.3 The initial report was of two siblings and their mother all of whom displayed persistently short QT interval. The youngest was a 17 year old female presenting with several episodes of paroxysmal atrial fibrillation requiring electrical cardioversion.3 Her QT interval measured 280 msec at a heart rate of 69. Her 21 year old brother displayed a QT interval of 272 msec at a heart rate of 58, whereas the 51 year old mother showed a QT of 260 msec at a heart rate of 74. The authors also noted similar ECG findings in another unrelated 37 year old patient associated with sudden cardiac death.http://www.ipej.org/0402/antzelevitch.htmshort QT syndromegenetic studies |
| spellingShingle | Charles Antzelevitch Johnson Francis Congenital Short QT Syndrome Indian Pacing and Electrophysiology Journal short QT syndrome genetic studies |
| title | Congenital Short QT Syndrome |
| title_full | Congenital Short QT Syndrome |
| title_fullStr | Congenital Short QT Syndrome |
| title_full_unstemmed | Congenital Short QT Syndrome |
| title_short | Congenital Short QT Syndrome |
| title_sort | congenital short qt syndrome |
| topic | short QT syndrome genetic studies |
| url | http://www.ipej.org/0402/antzelevitch.htm |
| work_keys_str_mv | AT charlesantzelevitch congenitalshortqtsyndrome AT johnsonfrancis congenitalshortqtsyndrome |