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Precision medicine for long QT syndrome in children

??Long QT syndrome??LQTS?? is the first found genetic ion channel disease. LQTS patients can show various clinical types?? from a lifetime of being asymptomatic to infantile sudden death?? which can cause malignant ventricular arrhythmia?? syncope?? epileptic seizures?? cardiac arrest and sudden cardiac death. The genetics of LQTS pathogenesis is currently considered specific genetic mutations that can lead to abnormal procedure of depolarization and repolarization of cardiac muscle cell. At the molecular level?? the pathogenesis of LQTS is the 15 different susceptible gene mutations of alpha and beta subunit that encoded ion channels. KCNQ1??KCNH2 and SCN5A mutations account for more than 90% of all mutations in patients with LQTS??and the remained 12 mutations account for only less than 10%. Precision medicine for LQTS mainly lies in the application of beta receptor blockers. Currently it’s believed that beta receptor blockers is better in the treatment of LQTS1 than in LQTS2 and LQTS3. Patients with LQT3 have low beta adrenergic receptor density?? and attacks are mainly related to rest and slow heart rate. Sodium channel blockers are more often recommended in clinical treatment??such as mexiletine??fluorine carney??etc.