Korean Circ J.  1998 Nov;28(11):1882-1888. 10.4070/kcj.1998.28.11.1882.

A Case of Congenital Long QT Syndrome Associated with Deafness and Syncope

• Lee SM
• Choe CW
• Kang HS
• Kim KS
• Song JS
• Bae JH

• KMID: 1858300
• DOI: http://doi.org/10.4070/kcj.1998.28.11.1882

Abstract

Congenital long QT syndrome (LQTS) is an inherited disease characterized by prolonged QT intervals and polymorphic ventricular tachycardia. The clinical manifestations vary from sudden cardiac death by ventricular arrhythmia to asymptom throughout life. In 1957, Jervell and Lange-Nielsen reported a syndrome of congen-ital sensory deafness associated with a prolonged QT interval in four children. The affected children had multiple syncopal episodes, and three died suddenly. The mode of inheritance is autosomal recessive. Affected persons are susceptible to recurrent syncope, and they have a high incidence of sudden death and short life expectancy. We report a case and review the literature on long QT syndrome diagnosed in a 30-year-old female with a history of convulsion and loss of consciousness during delivery.

Keyword

Congenital long QT syndrome; Jervell and Lange-Nielsen syndrome

MeSH Terms

Adult
Arrhythmias, Cardiac
Child
Deafness*
Death, Sudden
Death, Sudden, Cardiac
Female
Humans
Incidence
Jervell-Lange Nielsen Syndrome
Life Expectancy
Long QT Syndrome*
Seizures
Syncope*
Tachycardia, Ventricular
Unconsciousness
Wills