Abstract 16827: Double-chambered Right Ventricle Diagnosed in Late Adulthood by Multi-modality Imaging. Report of Two Cases With Associated Aortopathy

Introduction: Double-chambered right ventricle (DCRV) is a rare congenital disorder. The right ventricle (RV) is divided into two: a high-pressure inlet and a low-pressure outlet by an abnormal muscle bundle. Rarely diagnosed in adulthood, it is associated with other congenital defects. Association with aortopathy is not described. Patient 1: 67 year-old male with dyspnea: PE: grade 4 harsh systolic murmur radiating to the base. Echo: DCRV with obstruction -peak gradient (PG) 75mmHg (Fig 1-C), hypertrophied/dilated RV, decreased function; small perimembranous VSD, small right-to-left shunt. Ascending aorta aneurysm: 49 mm (Fig 1-D). Normal aortic valve. MRI: DCRV with muscle bundle causing RVOT obstruction. RV with delayed gadolinium enhancement (Fig 2). Heart cath: severe RV obstruction (PG 105 mmHg). Surgery: Resection of the muscular bundle, VSD closure and grafting of ascending aorta. Follow-up: significant improvement. Patient 2: 56 year-old female with VSD. PE: 4/6 holosystolic murmur at left sterna...