A complex of methods including clinical analysis, ECG, and exercise EEG was used to examine 52 patients aged 2 to 20 years with Romano-Ward syndrome. There were two groups: group I with syncopal attacks, group II without them. The patients with Romano-Ward syndrome had dysfunction of the suprasegmental systems regulated autonomously in the form of functional insufficiency of the mesencephalic activating system. In the patients without syncopal attacks, there was hyperproduction of the theta-rhythm, which may reflect the mechanisms of compensation. According to the EEG readings, criteria for the syndrome gravity and sudden death risk factors were distinguished.
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J Cardiovasc Electrophysiol
January 2025
Department of Molecular Pharmacology & Experimental Therapeutics (Windland Smith Rice Sudden Death Genomics Laboratory), Mayo Clinic, Rochester, Minnesota.
Stem Cell Res
December 2024
Division of Cardiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, South Korea. Electronic address:
Circ Res
September 2024
Dr. Neher's Biophysics Laboratory for Innovative Drug Discovery, State Key Laboratory of Quality Research in Chinese Medicine, Macau University of Science and Technology, Taipa, Macao SAR, China (L.Z., Z.Y., D.J., Y.O., H.Z., X.L., C.X., C.H., B.S., S.K.C., Z.-H.J., E.N., P.H.).
Eur Heart J
September 2024
Translational Cardiology, Department of Cardiology and Department of Physiology, University Hospital Bern, University of Bern, Bühlplatz 5, 3012 Bern, Switzerland.
Background And Aims: Type 1 long QT syndrome (LQT1) is caused by pathogenic variants in the KCNQ1-encoded Kv7.1 potassium channels, which pathologically prolong ventricular action potential duration (APD). Herein, the pathologic phenotype in transgenic LQT1 rabbits is rescued using a novel KCNQ1 suppression-replacement (SupRep) gene therapy.
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