Screening Vital for Relatives of Long-QT Patients

June 27, 2008

It is becoming increasingly common for children to be identified with congenital long-QT syndrome because of family screening, and with appropriate therapy, survival is excellent among both probands and non-probands, according to a report published in the June 15 issue of the American Journal of Cardiology.

FRIDAY, June 27 (HealthDay News) -- It is becoming increasingly common for children to be identified with congenital long-QT syndrome because of family screening, and with appropriate therapy, survival is excellent among both probands and non-probands, according to a report published in the June 15 issue of the American Journal of Cardiology.

Colin Petko, M.D., of the University of Utah in Salt Lake City, and colleagues documented the clinical courses and outcomes of 144 pediatric patients with long-QT syndrome at three pediatric centers. Eighty-four of the patients were non-probands. Demographic data, personal and family histories, electrocardiographic data, and genetic diagnoses were obtained. Probands were defined as the first patients in their families diagnosed with long-QT syndrome, and non-probands as those diagnosed by screening.

During a follow-up period that averaged 4.7 years, the researchers report that there were no deaths in the non-proband group, but device implantation was performed in 13 non-proband patients (15 percent). Four of them had appropriate shocks. The investigators also found that non-probands were less symptomatic and younger than probands, and that they had shorter QTc intervals. There was no difference in appropriate implantable cardioverter-defibrillator shocks or mortality between the two groups.

"Having a proband as a first-degree relative did not confer an increased risk for symptoms compared with a more distant relative. Thus, continued follow-up throughout childhood is mandatory to adequately protect proband and non-proband patients with long-QT syndrome," the authors write.

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