Nonsurgical device closure of isolated ostium secundum atrial septal defect in a case of genetically proven tuberous sclerosis

Abstract

Tuberous sclerosis (TSC) is an autosomal dominant multisystem disease associated with multiple cardiac Rhabdomyomas; however, congenital cardiac malformations are very rare in TSC. A 5-year-old girl with classical features of TSC had an associated large secundum atrial septal defect (ASD). The left to right shunt through the ASD was augmented by the multiple left ventricular masses. Nonsurgical closure of the ASD avoided the neurological and cardiac complications that may occur due to cardiopulmonary bypass during open-heart surgery. This first novel interventional report in TSC stresses on the value of such catheter interventions tomitigate risks in complex cardiac associations.