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June 1993

Rhabdomyoma and Ventricular Preexcitation Syndrome: A Report of Two Cases and Review of Literature

Author Affiliations

From the Department of Pediatrics, James H. Quillen College of Medicine, East Tennessee State University, Johnson City.

Am J Dis Child. 1993;147(6):669-671. doi:10.1001/archpedi.1993.02160300075027

• 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.(AJDC. 1993;147:669-671)

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