Author + information
- Received January 23, 2013
- Accepted January 29, 2013
- Published online August 13, 2013.
- ∗Department of Pediatrics, Divisions of Pediatric Cardiology, Hackensack University Medical Center, Hackensack, New Jersey
- †Department of Surgery, Division of Cardiothoracic Surgery, Hackensack University Medical Center, Hackensack, New Jersey
- ‡Department of Medicine, Division of Cardiology, Hackensack University Medical Center, Hackensack, New Jersey
A 7-year-old with Noonan’s syndrome presented to our institution with hypertrophic cardiomyopathy phenotype. The magnetic resonance imaging demonstrated biventricular involvement (A, Online Video 1). Additional views documented severe right ventricular outflow tract (RVOT) hypertrophy (B, Online Video 2) with dynamic subvalvular stenosis (C, Online Video 3). The vacillating nature of the right ventricular (RV) aneurysm is apparent (D, Online Videos 4 and 5). Despite high-dose beta-blockers, the stress echocardiogram revealed the progressive nature of this aneurysm (E, Online Videos 6 and 7). To the best of our knowledge, this is the first reported case of hypertrophic obstructive cardiomyopathy with biventricular involvement and an RV aneurysm in Noonan’s syndrome. We previously reported an RV aneurysm in an adult with hypertrophic cardiomyopathy (1), but we believe this report is the first in a child. LV = left ventricular.
- Received January 23, 2013.
- Accepted January 29, 2013.
- American College of Cardiology Foundation