Abstract
common hereditary pattern for congenital cardiac lesions.“’ In the family presented, we would assume a low trigger threshold. According to Nora and Nora,‘O. ” given one sibling with TA, the recurrence risk of any congenital cardiac lesion in siblings is about l%, a low risk, with conotruncal anomalies predominating. The literature contains several reports of familial occurrence of conotruncal anomalies, but only one report of siblings with TA” and no reports of siblings with single ventricle. While the recurrence risk for future siblings is unknown, a risk above 25% is likely. Paliation in infancy, as planned for patient No. 2, was directed at maintenance of low PA pressure and low pulmonary vascular resistance allowing the potential for a Fontan-type procedure at a future time.
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