31-year old man with Short QT syndrome (RCD code: V-1A.3)

Sylwia Wiśniowska‑Śmiałek, Paweł Rubiś, Katarzyna Holcman, Barbara Biernacka‑‑Fijałkowska, Agata Leśniak‑Sobelga, Grzegorz Kopeć, Magdalena Kostkiewicz, Piotr Podolec

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Abstract


Short QT (SQTS) syndrome is a rare inherited autosomal dominant cardiac channelopathy associated with malignant ventricular and atrial arrhythmias. It is the severest form of the major channelopathies, with cardiac arrest or sudden cardiac death (SCD) as the most common presentation. We report a case of a young patient in whom ventricular fibrillation was the first manifestation of the disease.

Keywords


rare disease; channelopathy; electrocardiography; implantable cardioverter‑defibrillator

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DOI: http://dx.doi.org/10.20418%2Fjrcd.vol2no7.220

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