What is the definitive treatment for symptomatic long QT syndrome?

Symptomatic long QT syndrome is a condition that predisposes individuals to potentially fatal arrhythmias, such as Torsades de Pointes. The definitive treatment in symptomatic patients is the implantation of an automatic implantable cardioverter-defibrillator (AICD). This device continuously monitors the heart's rhythm and can deliver an electric shock if a dangerous arrhythmia occurs, thereby preventing sudden cardiac death.

While correcting electrolyte imbalances is essential in managing any patient with long QT syndrome, it does not address the underlying arrhythmogenic risk associated with the syndrome itself. Medications, although often used as first-line therapy for some types of long QT syndrome, may not effectively control symptoms in all patients, particularly those with severe and symptomatic cases. Vagal maneuvers are typically employed for acute intervention in certain arrhythmias rather than as a long-term management strategy, further emphasizing the need for a reliable preventive measure like the AICD in symptomatic cases.

Hence, the AICD is considered the definitive treatment because it provides immediate intervention in the event of life-threatening arrhythmias, significantly reducing the risk of sudden cardiac death in symptomatic individuals with long QT syndrome.