The Long QT Syndrome (L.Q.T.S) is a disorder inherited, rare, electric system of heart. It is with defects in the cellular structures of the muscle of  heart called channels of ions.
Heart is not implied in this anomaly, its function mechanical is normal.

These defects produce the lengthening of interval QT and support among patients a ground favourable with an abnormal acceleration of the rate of heartbeat (ventricular arrhythmia) called Torsades of points.

These serious disturbances involve a loss of  sudden conscience (syncope,poses cardiac) and can lead to sudden death by Fibrillation.

The long qt syndrome is responsible for many sudden deaths, in the young children, teenagers and young people adult.

LQTS can be inherited or acquired.

1. The inherited long QT Syndrome

It is necessary to distinguish two forms from inherited LQTS.

  1. The Long QT Syndrome of Jervell and Lange-Nielsen is rare, this form was described in 1957. It comprises severe symptoms, it is characterized by a deafness as of the birth. The syndrome Jervell and Lange-Nielsen is of recessive type autosomic inherited.

    -recessive Autosomique what wants to say that this hereditary transmission concerns the boys as well as the girls, but it is recessive since the disease must be transmitted by the father and the mother so that the child is reached.

  2. The second form of LQTS, Romano-Ward was described in 1963. Although badly known and still little identified, this form is more frequent, the patients do not have a problem of deafness. LQTS of Romano-Ward is bequeathed by dominant autosomic transmission.

    - Autosomique dominating means that the hereditary transmission is not related to the sex and that it is enough that a subject received abnormal gene of the one of his/her parents so that the disease appears. Each offspring of an affected relative has a chance on two to inherit mutant gene. In a family concerned with LQTS all the people can be free from this syndrome, if they do not inherit modified gene or all being affected or some of them without distinction sex and command birth.

2. The Long QT Syndrome acquired

This form is due to the catch of drugs. Many drugs are contra-indicated and present a danger to the patients reached of the SLQT. (see in the chapter concerning the list of the drugs.)

-Last update-august 29 2008-

Copyright2008 -Long QT syndrome association-