Noonan's Syndrome and Ebstein's Malformation of the Tricuspid Valve

IN THEIR review of noncardiac anomalies encountered in a large number of children with congenital heart disease, Noonan and Ehmke in 1963¹ noted a group of male and female children with pulmonic stenosis who also had stigmata suggestive of the Ullrich-Turner syndrome. In describing their syndrome, these authors included short stature, hypertelorism, mild mental retardation, congenital heart disease, ptosis, cryptorchidism, and various skeletal malformations. Summitt et al^2,3 further defined the syndrome and suggested that the eponym "Noonan's syndrome" be employed to designate these patients apart from the Ullrich-Turner syndrome. In their report of seven boys with Noonan's syndrome, these latter authors emphasized that Noonan's syndrome differs in several important respects from the Ullrich-Turner syndrome. These differences included the following characteristics: (1) a milder and more variable pattern of shortness of stature; (2) frequent occurrence of pulmonary valvular or arterial stenosis; (3) a greater incidence of mental retardation; (4) functioning fetal gonads which