THE NATURAL-HISTORY OF CARDIAC RHABDOMYOMA WITH AND WITHOUT TUBEROUS SCLEROSIS

The aim of the present study is to contribute to the knowledge of the natural history of cardiac rhabdomyoma in children with and without tu berous sclerosis. In a retrospective study, 33 children with cardiac r habdomyoma were collected from three pediatric cardiology centres. In 30/33 patients tuberous sclerosis was associated. High prevalence of c ardiac rhabdomyoma was found in infancy, with 21/23 detected before th e age of 1 year, and 11/33 before 1 month of age. Cardiac manifestatio ns were present in 19 patients: cardiac rhythm disturbances were detec ted in 13; in 6/33 a Wolff-Parkinson-White syndrome was documented, of which 4 presented paroxysmal arrhythmias. Obstructive or regurgitativ e phenomena were present in 5; and in 2 patients surgical removal prov ed necessary. With the exception of one tumoural mass in the right atr ium, all 77 tumours were located somewhere in the ventricles, includin g at atrio-ventricular valve level. Because of spontaneous regression of most of the tumoural masses, treatment should at first be symptomat ic, while surgical removal is required only in life-threatening condit ions, as documented in 2 of our 33 patients.