Short QT Syndrome and Drug Treatment: A systematic Literature Review and PRISMA Analysis

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Short QT Syndrome (SQTS) is a rare inherited myocardial ion channel disease characterized by abbreviated cardiac repolarization and shortened QT interval in ECGs, resulting to a high incidence of sudden death and malignant arrhythmias. While various gene mutations that encode subunits of K+, Ca2+, and Na+ channels, as well as the SLC4A3 gene mutation associated with plasma membrane anion exchange, have been implicated, targeted gene screening remains relatively low. In this review, we searched multiple databases, such as PubMed, ScienceDirect, Embase, Web of Science, and Medline, and followed the guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) to conduct a systematic review of literatures in SQTS. We first used VOSviewer to analyze the co-authorship, co-occurrence of countries, organizations, authors, and keywords in the published literatures of SQTs, and then surveyed evidences regarding the impact of single or polygenic gene mutations identified is SQT patients on the electrophysiological properties of IKr, IKs, IK1, ICa-L, INa, and the anion exchanger AE3. Additionally, this review also surveyed current progress in the understandings of potential mechanisms underlying arrhythmogenesis of the SQT gene mutations, and possible drug therapy, unraveled by both experimental and simulation studies.