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Transposition of the Great Arteries, Pulmonary Atresia, and Multiple Ventricular Septal Defects Associated with Multiple Cardiac Rhabdomyomas in a Case of Tuberous Sclerosis
Authors:Z.Y. Jiang  A. Pircova  N. Sekarski  I. Hack  R. Laurini  R. Janzer  M. Payot
Affiliation:(1) Department of Cardiology, Centre Hospitalier Universitaire Vaudois, 1011 Lausanne, Switzerland, CH;(2) Pediatric Cardiology Unit, Department of Pediatrics, Centre Hospitalier Universitaire Vaudois, 1011 Lausanne, Switzerland, CH;(3) Developmental and Pediatric Pathology, Centre Hospitalier Universitaire Vaudois, 1011 Lausanne, Switzerland, CH;(4) Neuropathology, Institute of Pathology, Centre Hospitalier Universitaire Vaudois, 1011 Lausanne, Switzerland, CH
Abstract:Multiple cardiac rhabdomyomas are frequently associated with tuberous sclerosis. However, very few cardiac malformations have been described with tuberous sclerosis. We report an unusual case of tuberous sclerosis with cerebral and renal lesions associated with dextrotransposition of the great arteries, pulmonary atresia, patent ductus arteriosus, multiple ventricular septal defects, and parachute mitral valve deformity. After reviewing other cases of congenital heart diseases associated with tuberous sclerosis, emphasis is made on the potential influence of multiple rhabdomyomas developing very early during fetal life on final cardiac structures.
Keywords:: Cardiac rhabdomyomas —   Transposition of the great arteries —   Pulmonary atresia —   Congenital heart diseases —   Tuberous sclerosis
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