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Outcome positive for patients with long QT syndrome
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Despite a historically high-mortality rate in patients with long QT syndrome and 2:1 atrioventricular block, researchers observed no mortality among patients with these two conditions.
The U.S. researchers performed a retrospective study in four tertiary care pediatric centers, assessing patients with congenital long QT syndrome (LQTS) and 2:1 atrioventricular block (AVB) from January 2000 to January 2009. Researchers reviewed medical records for electrocardiogram findings, genotype, medications and device therapy, and performed statistical analysis using a paired t-test.
The study population (n=12) underwent diagnostic ECG during the first 24 hours of life. The average QTc interval before therapy was 616 ± 99 ms (range 531–840 ms). During a 71 ± 45 month follow-up period, 11 of 12 patients were given devices, including eight permanent pacemakers and three implantable cardioverter defibrillators. All patients received beta-blockers as treatment, while researchers added mexiletine in three patients, and mexiletine and flecainide in one.
At the final follow-up, there was no reported mortality and follow up QTc intervals had decreased (mean 480 ± 20 ms, P<.002).
For the researchers, the findings illustrated a relatively optimistic prognosis for patients with congenital LQTS and 2:1 AVB in the current era of therapy. “As demonstrated in this study, prenatal and postnatal diagnosis allowed for appropriate monitoring and early intervention,” they wrote. “This finding, coupled with an understanding of genotype-phenotype correlations as well as advances in device technology and surgical techniques, allows for administration of lifesaving care to a high-risk patient population.”
Aziz P. Heart Rhythm. 2010;7:781-785.