title: Massive global right ventricular hypertrophy with both fixed and dynamic obstruction and pulmonary valve dysplasia.
abstract: 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.
authors.affiliation: Department of Medicine, Division of Cardiology, Washington University School of Medicine, 660 S. Euclid Avenue, Saint Louis, MO 63110, USA. acedars@dom.wustl.edu
authors.forename: Ari M
authors.initials: AM
authors.lastname: Cedars
authors.forename: Cylen
authors.initials: C
authors.lastname: Javidan-Nejad
authors.forename: Manu
authors.initials: M
authors.lastname: Goyal
authors.forename: Joseph
authors.initials: J
authors.lastname: Billadello
authors.forename: Philip
authors.initials: P
authors.lastname: Ludbrook
mesh_terms: D017023:Coronary Angiography; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000150:complications / Q000175:diagnosis; D017380:Hypertrophy, Right Ventricular / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011664:Pulmonary Valve / Q000002:abnormalities*; D014694:Ventricular Outflow Obstruction / Q000150:complications* / Q000175:diagnosis / Q000628:therapy