Massive Global Right Ventricular Hypertrophy with Both Fixed and Dynamic Obstruction and Pulmonary Valve Dysplasia

作者：Cedars Ari M^*; Javidan Nejad Cylen; Goyal Manu; Billadello Joseph; Ludbrook Philip

来源：Congenital Heart Disease, 2012, 7(1): 66-70.

DOI：10.1111/j.1747-0803.2011.00538.x

• Pulmonary Valve Dysplasia
• Noonan Syndrome
• Hypertrophic Cardiomyopathy
• Right Ventricular Hypertrophy
• Dynamic Right Ventricular Outflow Tract Obstruction

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.

• 出版日期2012-2