Double-chambered right ventricle in an adult with Noonan syndrome

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.