Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome?
Andersen-Tawil syndrome (ATS) is a disorder that causes episodes of muscle weakness (periodic paralysis), changes in heart rhythm, and developmental abnormalities. QT prolongation and ventricular arrhythmias, including bidirectional ventricular tachycardia (VT) and polymorphic VT, may occur. About 6...
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| Format: | Article |
| Language: | English |
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KARE Publishing
2018-11-01
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| Series: | Türk Kardiyoloji Derneği Arşivi |
| Subjects: | |
| Online Access: | https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tkd&un=TKDA-77856 |
| _version_ | 1797906936019025920 |
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| author | Yakup Ergül Senem Özgür Sertaç Hanedan Onan Volkan Tuzcu |
| author_facet | Yakup Ergül Senem Özgür Sertaç Hanedan Onan Volkan Tuzcu |
| author_sort | Yakup Ergül |
| collection | DOAJ |
| description | Andersen-Tawil syndrome (ATS) is a disorder that causes episodes of muscle weakness (periodic paralysis), changes in heart rhythm, and developmental abnormalities. QT prolongation and ventricular arrhythmias, including bidirectional ventricular tachycardia (VT) and polymorphic VT, may occur. About 60% of all cases of the disorder are caused by mutations in the KCNJ2 gene. A 13-year-old female patient was referred for frequent premature ventricular contractions. Suspicion of ATS due to dysmorphic findings, electrocardiogram changes, and periodic muscle weakness was genetically confirmed. Beta-blocker therapy was initiated as a first-line treatment for bidirectional VT and frequent polymorphic premature ventricular contractions. Despite proper treatment, the VT attacks were not brought under control. Flecainide was added to the treatment regime. The number of premature ventricular contractions was dramatically reduced with flecainide and the VT attacks completely disappeared. This patient is a rare example of ATS in our country. This article provides a description of successful management of rhythm disturbance in a patient with ATS |
| first_indexed | 2024-04-10T10:28:34Z |
| format | Article |
| id | doaj.art-37ce2ed10896435eaf263d75a6ee68f3 |
| institution | Directory Open Access Journal |
| issn | 1016-5169 |
| language | English |
| last_indexed | 2024-04-10T10:28:34Z |
| publishDate | 2018-11-01 |
| publisher | KARE Publishing |
| record_format | Article |
| series | Türk Kardiyoloji Derneği Arşivi |
| spelling | doaj.art-37ce2ed10896435eaf263d75a6ee68f32023-02-15T16:21:13ZengKARE PublishingTürk Kardiyoloji Derneği Arşivi1016-51692018-11-0146871872210.5543/tkda.2017.77856TKDA-77856Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome?Yakup Ergül0Senem Özgür1Sertaç Hanedan Onan2Volkan Tuzcu3Department of Pediatric Cardiology and Arrhythmia, Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, TurkeyDepartment of Pediatric Cardiology and Arrhythmia, Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, TurkeyDepartment of Pediatric Cardiology, Bağcılar Training and Research Hospital, İstanbul, TurkeyDepartment of Pediatric Cardiology and Arrhythmia, Medipol University Hospital, İstanbul, TurkeyAndersen-Tawil syndrome (ATS) is a disorder that causes episodes of muscle weakness (periodic paralysis), changes in heart rhythm, and developmental abnormalities. QT prolongation and ventricular arrhythmias, including bidirectional ventricular tachycardia (VT) and polymorphic VT, may occur. About 60% of all cases of the disorder are caused by mutations in the KCNJ2 gene. A 13-year-old female patient was referred for frequent premature ventricular contractions. Suspicion of ATS due to dysmorphic findings, electrocardiogram changes, and periodic muscle weakness was genetically confirmed. Beta-blocker therapy was initiated as a first-line treatment for bidirectional VT and frequent polymorphic premature ventricular contractions. Despite proper treatment, the VT attacks were not brought under control. Flecainide was added to the treatment regime. The number of premature ventricular contractions was dramatically reduced with flecainide and the VT attacks completely disappeared. This patient is a rare example of ATS in our country. This article provides a description of successful management of rhythm disturbance in a patient with ATShttps://jag.journalagent.com/z4/download_fulltext.asp?pdir=tkd&un=TKDA-77856bidirectional ventricular tachycardiaflecainide; long qt. |
| spellingShingle | Yakup Ergül Senem Özgür Sertaç Hanedan Onan Volkan Tuzcu Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome? Türk Kardiyoloji Derneği Arşivi bidirectional ventricular tachycardia flecainide; long qt. |
| title | Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome? |
| title_full | Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome? |
| title_fullStr | Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome? |
| title_full_unstemmed | Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome? |
| title_short | Can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with Andersen-Tawil syndrome? |
| title_sort | can flecainide totally eliminate bidirectional ventricular tachycardia in pediatric patients with andersen tawil syndrome |
| topic | bidirectional ventricular tachycardia flecainide; long qt. |
| url | https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tkd&un=TKDA-77856 |
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