title: The natural history of cardiac rhabdomyoma with and without tuberous sclerosis.
abstract: The aim of the present study is to contribute to the knowledge of the natural history of cardiac rhabdomyoma in children with and without tuberous sclerosis. In a retrospective study, 33 children with cardiac rhabdomyoma were collected from three pediatric cardiology centres. In 30/33 patients tuberous sclerosis was associated. High prevalence of cardiac rhabdomyoma was found in infancy, with 21/23 detected before the age of 1 year, and 11/33 before 1 month of age. Cardiac manifestations were present in 19 patients: cardiac rhythm disturbances were detected in 13; in 6/33 a Wolff-Parkinson-White syndrome was documented, of which 4 presented paroxysmal arrhythmias. Obstructive or regurgitative phenomena were present in 5; and in 2 patients surgical removal proved necessary. With the exception of one tumoural mass in the right atrium, all 77 tumours were located somewhere in the ventricles, including at atrio-ventricular valve level. Because of spontaneous regression of most of the tumoural masses, treatment should at first be symptomatic, while surgical removal is required only in life-threatening conditions, as documented in 2 of our 33 patients.
authors.affiliation: Pediatric Cardiology Unit, University of Ferrara, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Bosi
authors.forename: J P
authors.initials: JP
authors.lastname: Lintermans
authors.forename: P A
authors.initials: PA
authors.lastname: Pellegrino
authors.forename: G
authors.initials: G
authors.lastname: Svaluto-Moreolo
authors.forename: A
authors.initials: A
authors.lastname: Vliers
mesh_terms: D001145:Arrhythmias, Cardiac / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006338:Heart Neoplasms / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000503:physiopathology; D012075:Remission, Spontaneous; D012189:Retrospective Studies; D012207:Rhabdomyoma / Q000150:complications* / Q000503:physiopathology; D014402:Tuberous Sclerosis / Q000150:complications*