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October 1968

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

Author Affiliations

(Canada); Memphis, Tenn
From the sections of cardiology (Drs. Wright and Ainger) and genetics (Dr. Summitt), Department of Pediatrics, University of Tennessee College of Medicine, the Variety Children's Heart Institute, and the Frank T. Tobey Memorial Children's Hospital, Memphis, Tenn.

Am J Dis Child. 1968;116(4):367-372. doi:10.1001/archpedi.1968.02100020371004

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 al2,3 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

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