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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2021.1
- 수록면
- 59 - 63 (5page)
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초록· 키워드
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An important, albeit rare, cause of fetal bradycardia is long QT syndrome (LQTS). Congenital LQTS is an ion channelopathy caused by mutations in genes encoding cardiac ion channel proteins. Fetal onset of LQTS imposes high risk of life-threatening tachyarrhythmias and sudden cardiac death. Here, we report the case of a female newborn with fetal onset of bradycardia and a 2:1 atrioventricular (AV) block. After birth, a 12-lead electrocardiogram (ECG) revealed bradycardia with QT prolongation of a corrected QT (QTc) interval of 680 ms and pseudo 2:1 AV block. Genetic testing identified a heterozygous Gly402Ser (c.1204G>A) mutation in CACNA1C, confirming the diagnosis of LQTS type 8 (LQT8). The patient received propranolol at a daily dose of 2 mg/kg. Mexiletine was subsequently administered owing to the sustained prolongation of the QT interval and pseudo 2:1 AV block. One week after mexiletine inception, the ECG still showed QT interval prolongation (QTc, 632 ms), but no AV block was observed. There were no life-threatening tachyarrhythmias in a follow-up period of 13 months.
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등록된 정보가 없습니다.
참고문헌 (14)
참고문헌 신청
Maeno Y / 2009 / Fetal arrhythmia : prenatal diagnosis and perinatal management / J Obstet Gynaecol Res 35:623~629
Anuwutnavin S / 2013 / Fetal long QT syndrome manifested as atrioventricular block and ventricular tachycardia : a case report and a review of the literature / Pediatr Cardiol 34:1955~1962
Schwartz PJ / 2013 / Impact of genetics on the clinical management of channelopathies / J Am Coll Cardiol 62:169~180
Skinner JR / 2019 / Channelopathies that lead to sudden cardiac death : clinical and genetic aspects / Heart Lung Circ 28:22~30
Splawski I / 2004 / Ca(V)1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism / Cell 119:19~31
Anuwutnavin S / 2013 / Fetal long QT syndrome manifested as atrioventricular block and ventricular tachycardia : a case report and a review of the literature / Pediatr Cardiol 34:1955~1962
Schwartz PJ / 2013 / Impact of genetics on the clinical management of channelopathies / J Am Coll Cardiol 62:169~180
Skinner JR / 2019 / Channelopathies that lead to sudden cardiac death : clinical and genetic aspects / Heart Lung Circ 28:22~30
Splawski I / 2004 / Ca(V)1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism / Cell 119:19~31
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