The history of research and the evolution of views on long QT syndrome. A Literature Review

Long QT syndrome (LQTS) is a heterogeneous hereditary or acquired myocardial repolarization disorder associated with a high risk of torsades de pointes ventricular arrhythmia and sudden cardiac death. This review traces the evolution of scientific understanding of LQTS from the first clinical descriptions in the 1950s to modern advances in molecular genetics. Particular attention is paid to key stages in the pathophysiological conceptualization of the syndrome, including the role of the sympathetic nervous system, T-wave alternans, the introduction of β-blockers and left cardiac sympathetic denervation, as well as the identification of genes responsible for various LQTS subtypes (KCNQ1, KCNH2, SCN5A, etc.). Current research directions are reviewed: the use of induced pluripotent stem cells, gene correction methods (siRNA, CRISPR/Cas9), and artificial intelligence for diagnosis and risk stratification. The contribution of domestic researchers to the development of clinical and genetic approaches to studying the syndrome is noted. The accumulated experience indicates a transition from empirical therapy to personalized management of patients with LQTS and outlines prospects for further research in the areas of genetic modifiers, pathogenetic treatments, and digital technologies in cardiology.

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