EVOLUTION OF CARDIAC RHABDOMYOMA IN TUBEROUS SCLEROSIS COMPLEX

The objective of the study was to define the longitudinal evolution of cardiac rhabdomyomas (CR) in patients with tuberous sclerosis complex (TSC). A cohort of patients with TSC who had undergone videotaped ech ocardiographic (ECHO) examination during the 10-year interval (1984-19 94) were retrospectively studied by reviewing and quantifying the CR a ppearance and associated cardiac abnormalities in sequentially obtaine d ECHO examinations. Sixteen patients with TSC (8 males) underwent a t otal of 35 recorded studies. Ten of the 16; (62.5%) had CR identified at initial study; none were found in the atria. Localization was the v entricular walls was compared with the ventricular septum by a ratio o f 2:1. The number of CRs sequentially studied declined as follows; ini tial study: 23 lesions in 10 patients; second study: 16 lesions in 8 p atients; third study: 12 lesions in 5 patients; and fourth study: 4 le sions in 2 patients. Total CR size index declined at each study as fol lows: initial index of 2,684; second index of 1,746 (-35% from initial ); third index 1,141 (-57% from initial); and fourth index 705 (-74% f rom initial). Complete spontaneous regression of CR was seen by age 6 years with prolonged gradual resolution thereafter. Two patients had b icuspid aortic valves and two had conduction defects. Patients with TS C who have CR can be expected to experience a decline in both the numb er and size of CR over time; early complete regression on ECHO occurs before age 6 years.