Acquired right ventricular outflow obstruction in a child with neurofibromatosis

A case of severe acquired localized right ventricular obstruction in an 8 1 2-year-old child with von Recklinghausen's syndrome is presented. The similarity of this case to vascular involvement in previously reported cases is pointed out. It is suggested that vascular lesions in neurofibromatosis may have varied manifestations and that this syndrome should be thought of in the future as a generalized disease which may affect any part of the cardiovascular system. For the lesion here reported, excision of the fibromuscular band has resulted in disappearance of her pressure difference, murmur, and right heart failure.