Congenital pulmonary valvular dysplasia is an unusual condition typically associated with Noonan syndrome. Among its other cardiac manifestations, occasional patients with Noonan syndrome have been demonstrated to have hypertrophic nonobstructive cardiomyopathy, which may be biventricular. We report a unique case of pulmonary valvular dysplasia, dynamic right ventricular outflow tract obstruction and profound right ventricular hypertrophy with only mild left ventricular hypertrophy, in a patient without Noonan syndrome.
- Dynamic right ventricular outflow tract obstruction
- Hypertrophic cardiomyopathy
- Noonan syndrome
- Pulmonary valve dysplasia
- Right ventricular hypertrophy