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.