Rhabdomyoma and ventricular preexcitation syndrome. A report of two cases and review of literature
A. V. Mehta
Department of Pediatrics, James H. Quillen College of Medicine, East Tennessee State University, Johnson City 37614-0578.
OBJECTIVE--A description of two infants with ventricular preexcitation
syndrome and supraventricular tachycardia associated with rhabdomyoma and
review of the literature. DESIGN--A review of two patients who were seen
between 1981 and 1986 in the cardiology department. SETTING--Pediatric
Heart Institute at St Christopher's Hospital for Children, Philadelphia,
Pa. PARTICIPANTS--Two newborn female infants with ventricular preexcitation
syndrome, supraventricular tachycardia, rhabdomyomatous tumor of the heart,
and tuberous sclerosis are described. RESULTS--The first patient had
unsuccessful partial resection of the rhabdomyomatous tumors obstructing
the tricuspid valve and right ventricular cavity and died immediately after
surgery. By histologic examination, no direct accessory connection was
noted between the myocardial fibers of atria and ventricles through annulus
fibrosus. By gross examination, the tumor extended from the right atrium
through the tricuspid valve to the right ventricular cavity, suggestive of
macroscopic accessory connection. The second patient presented with
unsustained ventricular tachycardia and obstructive subaortic rhabdomyoma,
requiring emergency surgery. One week later, reentrant supraventricular
tachycardia developed and she required digoxin therapy for 15 months.
CONCLUSIONS--Infants with rhabdomyomatous tumor of the heart and
ventricular preexcitation syndrome may have microscopic or macroscopic
accessory connections. Cardiac tumors like rhabdomyoma and oncocytic tumors
should be suspected in infants with ventricular preexcitation syndrome or
supraventricular tachycardia.
