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Children and Adolescent Patients with the Variants in the ATP1A3-encoded Sodium-Potassium ATPase Alpha-3 Subunit Demonstrate Impaired QT Response to Bradycardia and Predisposition to Sinus Node Dysfunction
2024-09-01
cardiovascular medicine
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BackgroundAlternating hemiplegia of childhood (AHC) is a rare disorder with both neurologic and cardiac manifestations. The ATP1A3-D801N variant is associated with a pathologically short QT interval and risk of ventricular arrhythmia following bradycardia; however, the mechanism of this remains unknown. We investigated the relationship between heart rate (HR), QT, and QTc, hypothesizing that individuals with ATP1A3-D801N have abnormal, impaired shortening of QT and QTc at lower HR leading to arr...
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