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Hypoplastic Heart Syndrome Highly Heritable

Leading cause of infant death due to cardiovascular malformation

WEDNESDAY, Oct. 10 (HealthDay News) -- Hypoplastic left heart syndrome (HLHS), a leading cause of infant death due to cardiovascular malformation, is highly heritable, according to research published online Oct. 1 in the Journal of the American College of Cardiology.

D. Woodrow Benson, M.D., Ph.D., and colleagues from Cincinnati Children's Hospital Medical Center, performed electrocardiograms on 235 members of 38 families with an HLHS proband.

The researchers found that all probands had aortic valve hypoplasia and dysplasia. More than one individual was affected in 55 percent of families and 36 percent of subjects had cardiovascular malformation. The heritability of HLHS alone was 99 percent and with cardiac malformation was 74 percent.

"The high heritability of HLHS suggests that it is determined largely by genetic factors," Benson and colleagues conclude. "The frequent occurrence of left- and right-sided valve dysplasia in HLHS probands and the increased prevalence of bicuspid aortic valve in family members suggests that HLHS is a severe form of valve malformation."

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