FAMILIAL TOTAL ANOMALOUS PULMONARY VENOUS RETURN - A LARGE UTAH-IDAHOFAMILY

Total anomalous pulmonary venous return (TAPVR) is a rare form of cyan otic congenital heart disease which, without surgical correction, has a high mortality rate in the first year of life. It usually occurs wit hout a family history, and is generally thought to have a low recurren ce risk. However, 15 instances of familial TAPVR have been reported pr eviously, suggesting a genetic cause in some cases. We report on a lar ge family in which TAPVR appears to be determined multifactorially or as an autosomal dominant trait with variable expressivity and reduced penetrance. (C) 1994 Wiley-Liss, Inc.