RT Book, Section A1 O’Gara, Patrick T. A1 Loscalzo, Joseph A2 Jameson, J. Larry A2 Fauci, Anthony S. A2 Kasper, Dennis L. A2 Hauser, Stephen L. A2 Longo, Dan L. A2 Loscalzo, Joseph SR Print(0) ID 1155974464 T1 Pulmonic Valve Disease T2 Harrison's Principles of Internal Medicine, 20e YR 2018 FD 2018 PB McGraw-Hill Education PP New York, NY SN 9781259644016 LK accessmedicine.mhmedical.com/content.aspx?aid=1155974464 RD 2024/04/18 AB Pulmonic valve stenosis (PS) is essentially a congenital disorder (Table 262-1). With isolated PS, the valve is typically domed. Dysplastic pulmonic valves are seen as part of the Noonan syndrome (Chap. 275), which maps to chromosome 12. Mutations in the PTPN1 gene are associated with about half of all cases of Noonan syndrome. Much less common etiologies include carcinoid and obstructing tumors or bulky vegetations. The pulmonic valve is only very rarely affected by the rheumatic process.