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Updated: Aug 5, 2026

Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
Double-chambered right ventricle in an adult with Noonan syndrome
Anshul M Patel1, Jae Bum Kim, Amy E Roberts
1Cardiology Division, Massachusetts General Hospital, Boston, Massachusetts 02114, USA. ampatel@partners.org
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.
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