Congenital papillary tumor of the tricuspid valve. An unusual cause of right ventricular outflow obstruction in a neonate with trisomy E.

A trisomy E neonate had congenital cardiac anomalies that included a ventricular septal defect, a bicuspid aortic valve, and a congenital papillary tumor of the tricuspid valve. The large papillary tumor was responsible for severe intermittent pulmonary outflow obstruction. The cause and pathogenesis of these rare papillary tumors are unknown, but they are probably related to the more sessile varieties of congenital valvular dysplasia. The life-threatening obstruction to blood flow caused by the congenital valvular tumor probably can be alleviated by simple surgical resection. This type of tumor should be considered in the differential diagnosis of atypical forms of outflow tract obstruction.