Case ReportDouble-Chambered Right Ventricle in an Adult With Noonan SyndromePatel, Anshul M. MD*; Kim, Jae Bum MD†; Roberts, Amy E. MD‡; Aragam, Jayashri MD§Author Information From the *Cardiology Division, Massachusetts General Hospital, Boston, Massachusetts; the †Cardiovascular Medicine Division, Brigham & Women's Hospital, Boston, Massachusetts; the ‡Harvard Center for Genetics and Genomics, Boston, Massachusetts; and the §Cardiology Division, VA Boston Healthcare System, West Roxbury Campus, West Roxbury, Massachusetts. Reprints: Anshul M. Patel, MD, Cardiology Division, Bigelow 804, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114. E-mail: [email protected]. Cardiology in Review: September 2006 - Volume 14 - Issue 5 - p e16-e20 doi: 10.1097/01.crd.0000194163.60428.c0 Buy Metrics Abstract We report the case of a double-chambered right ventricle in a 59-year-old man with previously undiagnosed Noonan syndrome. The patient presented with dyspnea on exertion and his transthoracic echocardiogram suggested a right ventricular outflow tract obstruction. Cardiac catheterization, cardiac magnetic resonance imaging, and transesophageal echocardiography revealed a hypertrophic muscle bundle dynamically obstructing right ventricular outflow. He was referred for operative repair of his double-chambered right ventricle and had successful patch expansion of his right ventricular outflow tract. In addition, he underwent a medical genetics evaluation that confirmed a new diagnosis of Noonan syndrome. To the best of our knowledge, this is the first report of a double-chambered right ventricle in an adult patient with Noonan syndrome. © 2006 Lippincott Williams & Wilkins, Inc.