We sought to assess the spectrum and outcome of young long QT syndrome (LQTS) patients, addressing treatment including device indications, risks, and benefits.
Long QT syndrome has a phenotype ranging from asymptomatic electrocardiogram (ECG) abnormalities to sudden death. Treatments include beta-blockers and device implantation in high-risk individuals. Despite genetic testing, accurate risk stratification remains challenging.
A database search at 3 institutions identified all pediatric LQTS patients. Records were reviewed for demographics, criteria for diagnosis, treatment, follow-up, and ECG and device data.
We identified 128 patients ages 8.0 +/- 5.4 years with QTc of 487 +/- 39 ms and follow-up of 4.4 +/- 3.5 years. Most were diagnosed because of an abnormal ECG in a patient with a family history (53%). Genetic mutations were identified in 51 patients. Beta-blockers were used in 126 (98%) and pacemaker/implantable cardioverter-defibrillator implantation in 27 (21%) patients, usually because of symptoms despite use of beta-blockers. Pacing was common; 22% received an appropriate shock but device-related re-intervention occurred in 48%. Device patients had longer QTc intervals (p = 0.03) and more symptoms (p
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Comment In: J Am Coll Cardiol. 2007 Oct 2;50(14):1341-217903633