Why the study?
Does the combination of ICD parameter adjustment and propafenone prevent malignant arrhythmias in a child with AKAP9 mutation-associated long QT syndrome?
Does the combination of ICD parameter adjustment and propafenone prevent malignant arrhythmias in a child with AKAP9 mutation-associated long QT syndrome?
Rational ICD parameter settings combined with antiarrhythmic drugs like propafenone can effectively manage frequent electrical storms in children with AKAP9 mutation-associated long QT syndrome.
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May inform individualized ICD-drug strategies in rare pediatric AKAP9-LQTS; leaves open generalizability pending larger studies.
Introduction: gene, which is a controversial gene in long QT syndrome. After treatment with propranolol, recurrent syncope occurred, and the patient received an implantable cardioverter defibrillator (ICD). Due to frequent electrical storms at home, the child was additionally treated with propafenone to prevent arrhythmia. The antitachycardia pacing (ATP) function in the ICD was turned off, and the threshold of ventricular tachycardia (VT) assessment was adjusted from 180 beats/min to 200 beats/min. The patient was followed up for 12 months without malignant arrhythmia and electric shock. Conclusion: Genetic testing may be a useful tool to determine the origin of channelopathy, but the results should be interpreted in combination with the actual situation. Rational parameter settings for the ICD and application of antiarrhythmic drugs can reduce the mortality rates of children.
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Wang et al. (2023) studied this question.
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