{"id":11938,"date":"2025-06-17T00:48:48","date_gmt":"2025-06-16T22:48:48","guid":{"rendered":"https:\/\/valintermed.com\/?p=11938"},"modified":"2025-06-17T00:48:48","modified_gmt":"2025-06-16T22:48:48","slug":"sindrom-udlinennogo-intervala-qt-3","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/long-qt-interval-syndrome-3\/","title":{"rendered":"Long QT syndrome 3"},"content":{"rendered":"
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Long QT syndrome type 3 (LQT3) is a hereditary disorder of myocardial repolarization characterized by abnormal prolongation of the QT interval on the electrocardiogram. This form of the syndrome is caused by mutations in the SCN5A gene encoding the alpha subunit of the cardiac sodium channel. The disease is particularly dangerous due to the high risk of developing polymorphic ventricular tachycardia of the "pirouette" type and sudden cardiac death, especially at a young age. The pathophysiological mechanism of LQT3 is associated with delayed closure of sodium channels, which leads to an increase in the inward sodium current during the repolarization phase and, as a result, to an extension of the action potential of cardiomyocytes.<\/p>\n

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