Mexiletine infusion challenge test for neonatal long QT syndrome with 2:1 atrioventricular block
نویسندگان
چکیده
منابع مشابه
Congenital long QT syndrome with functionally impaired atrioventricular conduction: successful treatment by mexiletine and propranolol.
Congenital long QT syndrome (LQTS) with atrioventricular block is a rare and malignant arrhythmia, which usually responds poorly to traditional beta-blocker therapy. We describe a male neonate with LQTS with ventricular tachycardia and 2:1 atrioventricular block. This patient's sister had similar presentation and died suddenly at the age of 8 months despite beta-blocker and pacemaker therapy. O...
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Heterozygous mutations in genes encoding cardiac ionic channel subunits KCNQ1, HERG, SCN5A, KCNE1, and KCNE2 are causally involved in the dominant form of long-QT syndrome (LQTS) while homozygous mutations in KCNQ1 and KCNE1 cause LQTS with or without congenital deafness. In addition, two homozygous HERG mutations have been associated with severe LQTS with functional atrioventricular conduction...
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he congenital long QT syndrome (LQTS) is characterized by abnormally prolonged ventricular repolarization, leading to QT prolongation on the ECG, with frequent development of the polymorphic ventricular tachycardia known as torsades de pointes (Tdp), syncope or sudden cardiac death.1,2 Most cases of congenital LQTS are caused by gene mutations in several ion channels and their interacting prote...
متن کاملLong QT syndrome: A therapeutic challenge
Congenital long QT syndrome (LQTS) is one of the most common cardiac channelopathies and is characterized by prolonged ventricular repolarization and life-threatening arrhythmias. The mortality is high among untreated patients. The identification of several LQTS genes has had a major impact on the management strategy for both patients and family members. An impressive genotype-phenotype correla...
متن کاملFEATURED CASE REPORT Long QT syndrome and life threatening arrhythmia in a newborn: molecular diagnosis and treatment response
Intrauterine and neonatal manifestations of congenital long QT syndrome are associated with a high cardiac risk, particularly when atrioventricular block and excessive QT prolongation (. 600 ms) are present. In a female newborn with these features, treatment with propranolol and mexiletine led to complete reduction of arrhythmia that was maintained 1.5 years later. High throughput genetic analy...
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ژورنال
عنوان ژورنال: Journal of Arrhythmia
سال: 2019
ISSN: 1880-4276
DOI: 10.1002/joa3.12209