ECG showing typical pattern of inherited Long QT syndrome (LQT1). A QT interval of >480 ms is considered abnormally long. Long QT syndrome (LQTS) is a condition affecting repolarization (relaxing) of the heart after a heartbeat, giving rise to an abnormally lengthy QT interval. [7] .

Feb 20, 2003 · Long QT syndrome (LQTS) is characterized by QT prolongation and T wave abnormalities on EKG. LQTS predisposes individuals to a significant risk of life-threatening arrhythmic events, especially in young individuals.

Long QT syndrome (LQTS) is a rare disorder of the heart’s electrical system that can lead to dangerous heart rhythms, fainting, and sudden cardiac arrest. At Stanford, our internationally renowned electrophysiologists have the skill and experience to provide superior care for this complex condition.

Jan 27, 2017 · Long QT syndrome (LQTS) is an autosomal dominant disorder, caused by abnormalities of the heart’s electrical conduction system, and is characterized on the electrocardiogram (a test that records the electrical activity of the heart) by prolongation of the QT interval that corresponds to prolongation of the recovery phase or repolarization of the...

In this review, we summarize the current understanding of LQTS and provide a practical framework for its diagnosis, risk stratification, and treatment with a focus on the 3 major autosomal dominant LQTS genotypes: LQT1, LQT2, and LQT3 (Central Illustration, Table 1).

Long QT syndrome type 1 (LQT1) is a subtype of a congenital cardiac syndrome caused by mutation in the KCNQ1 gene, which encodes the α- subunit of the slow component of delayed rectifier K + current (IKs) channel.

Oct 7, 2022 · More than 15 forms of LQTS have been described, but most cases fall into 3 subgroups: Long QT syndrome type 3 (LQTS3): Gain-of-function mutation of gene SCN5A, which encodes the Nav1.5 channel responsible for the inward sodium current (INa). The vast majority of cases are LQTS1, LQTS2, or LQTS3.

Aug 1, 2012 · LQTS is typically characterized by a prolongation of the QT interval on the ECG and by the occurrence of syncope or cardiac arrest, mainly precipitated by emotional or physical stress.

LQTS1 is the most common type of congenital LQTS, accounting for 30%-35% of all LQTS cases. KCNQ1 encodes α -subunit of voltage-gated potassium channel Kv7.1, generating slow delayed rectifier potassium currents (IKs) (Ref. 33 ).

Congenital long QT syndrome (LQTS) is characterised by heart rate corrected QT interval prolongation and life-threatening arrhythmias, leading to syncope and sudden death.