Transposition of the great arteries, pulmonary atresia, and multiple ventricular septal defects associated with multiple cardiac rhabdomyomas in a case of tuberous sclerosis

Multiple cardiac rhabdomyomas are frequently associated with tuberous scler osis. However, very few cardiac malformations have been described with tube rous sclerosis. We report an unusual case of tuberous sclerosis with cerebr al and renal lesions associated with dextrotransposition of the great arter ies, pulmonary atresia, patent ductus arteriosus, multiple ventricular sept al defects, and parachute mitral valve deformity. After reviewing other cas es of congenital heart diseases associated with tuberous sclerosis, emphasi s is made on the potential influence of multiple rhabdomyomas developing ve ry early during fetal life on final cardiac structures.