Romano-Ward syndrome


Romano-Ward syndrome is a genetic condition associated with a prolonged QT interval on the ECG. Unlike Jervell-Lange-Nielsen, there is no congenital hearing loss present in Romano-Ward syndrome.

A mutation of the cardiac potassium channel or sodium channel is responsible and can be from one of multiple genetic mutations relating to these channels. Treatment includes beta-blockers, however if syncope or sustained ventricular arrhythmias are present, an implantable cardioverter defibrillator (ICD) is indicated to prevent sudden cardiac death.

ProlongedQT1

Prolonged QT interval.