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Beta-blockers are known to be efficacious for treating long-QT syndrome, particularly in LQTS1 and LQTS2 patients. Yet, sudden death and other cardiac events still occur in patients on beta-blockers. To explore why beta-blockers are not always effective, these investigators examined the clinical circumstances of cardiac events recorded in four international databases of LQTS1 patients who were followed for a median of 10 years after initiation of treatment with a beta-blocker.
Of 216 genotyped LQTS1 patients, 157 (73%) had cardiac events before starting a beta-blocker, including 26 (12%) with cardiac arrest (CA); in many patients, the event presumably prompted the LQTS diagnosis or initiation of beta-blocker therapy. Administration of a QT-p…