Genetic Arrhythmia Clinic
The Arrhythmia Clinic at Children's Hospital & Medical Center is the only program in the state specializing in the diagnosis and treatment of genetic arrhythmias in children, adolescents and adults.
Genetically related arrhythmias or cardiomyopathies in children are rare. The exact incidence or frequency is not known. Genetically related or transmitted arrhythmias or cardiomyopathies are usually caused by a genetic problem in the DNA (mutation or variant) that can either be passed from one or both parents or occur as a new variant. Some of the more common conditions that can cause arrhythmia include long QT syndrome, arrhythmogenic right ventricular dysplasia or cardiomyopathy, Brugada's syndrome, short QT syndrome, catecholaminergic polymorphic (or bidirectional) ventricular tachycardia (CPVT) and idiopathic ventricular fibrillation. Arrhythmia can also occur in association with an abnormal heart muscle (cardiomyopathy) such as hypertrophic cardiomyopathy (HCM), familial dilated cardiomyopathy, left ventricular non-compaction (LVNC), or arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC). If your child is diagnosed with a genetic arrhythmia, it is likely someone else in your family also has genetic arrhythmia. Your entire family will be recommended for genetic testing and genetic counseling.
Diagnosis
Diagnosis of genetically related problems can be complex and involve tests such as ECGs, Holter monitoring, exercise stress tests, epinephrine challenge, echocardiography, magnetic resonance imaging (MRI) or computed tomography (CT scan), and signaled-averaged ECG (SAECG). In addition, a sample of blood may be needed to perform a genetic analysis, which looks for specific mutations or variants in the patient's DNA.
Treatment
Treatment of genetic arrhythmias involves one or a combination of observation, medications, nonsurgical procedures or implantation of an internal cardioverter defibrillator. Most patients with genetic arrhythmias are treated successfully with medications and some require implantation of a device.
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