Advances In Long QT Syndrome

Long QT syndrome (long QT syndrome, LQTS) is easy to producemalignant ventricular arrhythmias (especially torsades de pointes ventriculartachycardia, TdP), which cause syncope, epileptic seizure attack and cardiacarrest and sudden cardiac death (SCD). It can be diagnosised according tothe patient’s ECG manifestation with a combination of family history, andbe divided into congenital and acquired long QT syndrome.Congenital long QT syndrome(congenital or inherited long QT syndrome,CLQT) is a disease of genemutation lead to myocardial repolarization disorders, At the molecularlevel, its pathogenesis is mutations in15distinct LQTS-susceptibility genesthat encode ion channel α-subunit and β-subunit lead to depolarization andrepolarization abnormalities. Now,we has identified13autosomal dominantgenetic type and two autosomal recessive genetic type, The most commontype is the clinical LQT1, LQT2and LQT3. Its electrophysiologicalmechanisms are certain genetic mutations affect myocardial cellmembrane’s sodium channels, potassium channels, calcium channels ormembrane switches, the action potential and QT prolonged, leading to earlyafter depolarization (EAD) and triggered activity induced TdP. Different genotypes evoked different ways show distinct clinicalmanifestations, ECG, risk stratification, prognosis and treatment are alsodifferences.Acquired long QT syndrome (acquired long QT syndrome, aLQTs)often associated with cardiac disease, electrolyte or metabolic disordersabnormalities and drug. AS part of listed drugs evacuated the market due toits serious side effects on the heart, the acquired long QT syndrome isconcerned by more and more people.The clinical study ofdrugs in the drug safety regulations the heart has been implemented. Withthe further understanding of the disease, the diagnosis of hospital-acquiredlong QT syndrome, treatment has improved significantly.Acute onset of long QT syndrome should be immediately terminatedmalignant arrhythmia, remove the cause, prevent malignant arrhythmiaappear again. After remission,the patient should avoid all kinds of factors,and reduce the sympathetic nerve excitement so as to reduce the risk ofarrhythmia, improve and terminate life-threatening malignant arrhythmia,prevention of sudden death.