Short QT Syndrome

Short QT Syndrome

·       A gain in function of repolarization currents can result in a shortening of atrial and ventricular refractoriness and marked QT shortening on the surface ECG (Table 233-8). The T wave tends to be tall and peaked.

·       A QT interval <320 ms="" span="">is required to establish the diagnosis of this uncommon syndrome.

·       Mutations in the HERG, KvLQT1, and KCNJ2 genes have been identified.

·       Patients with the syndrome are predisposed to both

o   AF and VF.

·       ICD implantation is recommended.

o   Double counting of QRS and T waves may lead to inappropriate ICD shocks.

·       Drug therapy with quinidine

o   lengthen the QT interval and reduce the amplitude of the T wave.