Long QT syndrome is a congenital disorder characterized by a prolongation of the QT interval on electrocardiograms and a propensity to ventricular tachyarrhythmias, which may lead to syncope, cardiac arrest, or sudden death.
symptoms Long QT syndrome is usually diagnosed after a person has a cardiac event (eg, syncope, cardiac arrest). In some situations, this condition is diagnosed after a family member suddenly dies. In some individuals, the diagnosis is made when an electrocardiogram shows QT prolongation.
Treatment All patients with long QT syndrome (LQTS) should avoid drugs that prolong the QT interval or that reduce their serum potassium or magnesium level. Potassium and magnesium deficiency should be corrected. Although treating asymptomatic patients is somewhat controversial, a safe approach is to treat all patients with congenital LQTS because sudden cardiac death can be the first manifestation of LQTS. Beta-blockers are drugs of choice for patients with LQTS. The protective effect of beta-blockers is related to their adrenergic blockade, which diminishes the risk of cardiac arrhythmias. They may also reduce the QT interval in some patients.
Research The first member of a family to be identified with LQTS, the proband, was usually brought to medical attention because of a syncopal episode during childhood or teenage years. Probands (n = 328) were younger at first contact (age 21 +/- 15 years), more likely to be female (69%), and had a higher frequency of preenrollment syncope or cardiac arrest with resuscitation (80%), congenital deafness (7%), a resting heart rate less than 60 beats/min (31%), QTc greater than or equal to 0.50 sec1/2 (52%), and a history of ventricular tachyarrhythmia (47%) than other affected (n = 688) and unaffected (n = 1,004) family members. Arrhythmogenic syncope often occurred in association with acute physical, emotional, or auditory arousal. The syncopal episodes were frequently misinterpreted as a seizure disorder. By age 12 years, 50% of the probands had experienced at least one syncopal episode or death. The rates of postenrollment syncope (one or more episodes) and probable LQTS-related death (before age 50 years) for probands (n = 235; average follow-up 54 months per patient) were 5.0% per year and 0.9% per year, respectively; these event rates were considerably higher than those observed among affected and unaffected family members.
Stroke was located in the right MCA territory in 21 (64%) and in the left MCA territory in 12 (36%) patients. Patients with right and left MCA stroke were similar with respect to time interval between baseline and admission ECG recordings, positive history of heart disease, and left ventricular dimensions. Increase in heart rate-corrected QT interval (QTc) from baseline to admission was demonstrated to occur more often in patients with right (16 of 21; 76%) than in patients with left (3 of 12; 25%; P < .01) MCA stroke. ΔQTc between baseline and admission was significantly longer in patients with right (23 ± 23 milliseconds) than in patients with left (-11 ± 19 milliseconds; P < .0001) MCA stroke. Percent ΔQTc between baseline and admission was longer in patients with right (5.5% ± 5.5%) than in patients with left (-2.6% ± 4.7%; P < .001) MCA stroke.