A case is presented in which an exceptional electrical alternans of the TU wave occurred in association with the Romano-Ward syndrome. This appears to be the first reported example of this association. Ventricular fibrillation was documented during the syncopal attacks. There was no evidence of abnormality of the serum electrolytes.

BACKGROUND The congenital long-QT syndrome (LQTS) is a genetically heterogeneous disease characterized by prolonged ventricular repolarization and life-threatening arrhythmias. Mutations of the KVLQT1 gene, a cardiac potassium channel, generate two allelic diseases: the Romano-Ward syndrome, inherited as a dominant trait, and the Jervell and Lange-Nielsen syndrome, inherited as an autosomal rec...

Genetic counselling in the autosomal dominant condition of the Romano-Ward syndrome might be assumed to be relatively straightforward. The problems posed by consanguinity, deafness, and subclinical gene carriers in a pedigree with this condition have caused us to reevaluate this view. The diagnostic and management difficulties which may attend this potentially fatal condition are highlighted by...

Patients with hereditary long QT disorders--Romano-Ward Syndrome and Jervell Lange-Nielsen Syndrome--sometimes present with seizures due to cardiac tachyarrhythmias. Two such patients are presented, emphasizing diagnostic clues--syncope, seizures with onset in early life, precipitation of attacks by emotional or physical stress, positive family history of sudden death and/or seizures, normal EE...

a case o f the electrical alternans of t he tu wave and peri odic nega t ive u wave associated with c l ini cal symptoms , e lectrocardi ographic a nd postmortem findi ng s o f romano- ward syndrome has been pres ented. no e lec ~ r o l y te d ist urbance was found t o be r esponsible for t his exceptional l y rare situation . changes i n a-v conduct i on and left bu n• d ie branch block could ...

BACKGROUND Long-QT syndrome (LQTS) is a disorder of ventricular repolarization characterized by a prolonged QT interval, syncope, seizures, and sudden death. Recently, three forms of LQTS have been shown to result from mutations in potassium or sodium ion channel genes: KVLQT1 for LQT1, HERG for LQT2, and SCN5A for LQT3. IsK, an apparent potassium channel subunit encoded by KCNE1 on chromosome ...