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Evolution of Cardiac Rhabdomyoma in Tuberous Sclerosis Complex

Department of Pediatrics, Division of Pediatric Neurology at the University of Connecticut Health Center, Farmington, CT; Connecticut Children's Medical Center, Department of Pediatrics, Division of Pediatric Neurology, 282 Washington Avenue, Hartford, CT 06106

Daniel Diana, M.D.

Harris Leopold, M.D.

Leon Chameides, M.D.

Department of Pediatrics, Division of Pediatric Cardiology, at Hartford Hospital, Hartford, CT

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 echocardiographic (ECHO) examination during the 10-year interval (1984-1994) were retrospectively studied by reviewing and quantifying the CR appearance and associated cardiac abnormalities in sequentially obtained ECHO examinations. Sixteen patients with TSC (8 males) underwent a total 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 ventricular walls as compared with the ventricular septum by a ratio of 2:1. The number of CRs sequentially studied declined as follows; initial study: 23 lesions in 10 patients; second study: 16 lesions in 8 patients; third study: 12 lesions in 5 patients; and fourth study: 4 lesions in 2 patients. Total CR size index declined at each study as follows: 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% from initial). Complete spontaneous regression of CR was seen by age 6 years with prolonged gradual resolution thereafter. Two patients had bicuspid aortic valves and two had conduction defects. Patients with TSC who have CR can be expected to experience a decline in both the number and size of CR over time; early complete regression on ECHO occurs before age 6 years.

Clinical Pediatrics, Vol. 35, No. 12, 615-619 (1996)
DOI: 10.1177/000992289603501202


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