It is possible that the main title of the record Jervell as well as Lange-Nielsen Disorder is not the name you expected. Please check the synonyms listing to find the alternate name(s) and problem neighborhood(s) covered by this report.
- autosomal recessive long QT syndrome (LQTS)
- Jervell as well as Lange-Nielsen (JLNS)
- cardioauditory syndrome
- cardioauditory syndrome of Jervell as well as Lange-Nielsen
- deafness, genetic, as well as practical heart disease
- surdocardiac disorder
Jervell as well as Lange-Nielsen disorder (JLNS) is an unusual acquired problem identified by hearing problems present at birth (congenital) taking place in association with abnormalities influencing the electric system of the heart. The intensity of heart symptoms related to JLNS differs from case to case. Some people might have no apparent signs (asymptomatic); others could develop unusually raised heartbeats (tachyarrhythmias) leading to episodes of unconsciousness (syncope), heart attack, and potentially untimely end. Exercising, excitement, shock, or stress may cause the beginning of these signs and symptoms. Passing out during the abovementioned tasks is also a traditional indicator of JLNS. JLNS is usually spotted during early youth and is inherited as an autosomal recessive genetic disorder. More than half of the neglected situations of JLNS lead to fatality before the age of 15.
Anton Jervell and also Fred Lange-Nielsen offered the very first complete description of the electric conduction trouble in the heart called long QT syndrome (LQTS) in 1957. LQTS refers to the QT-interval determined on the electrocardiogram that indicates that the heart muscular tissue is taking much longer than common to reenergize between beats. It predisposes those affected to tachyarrhythmias called torsade de pointes (TdP) which results in syncope and also might trigger sudden heart fatality.