RHABDOMYOMA AND VENTRICULAR PREEXCITATION SYNDROME - A REPORT OF 2 CASES AND REVIEW OF LITERATURE

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 we re seen between 1981 and 1986 in the cardiology department. Setting.-P ediatric Heart Institute at St Christopher's Hospital for Children, Ph iladelphia, Pa. Participants.-Two newborn female infants with ventricu lar preexcitation syndrome, supraventricular tachycardia, rhabdomyomat ous tumor of the heart, and tuberous sclerosis are described. Results. -The first patient had unsuccessful partial resection of the rhabdomyo matous tumors obstructing the tricuspid valve and right ventricular ca vity and died immediately after surgery. By histologic examination, no direct accessory connection was noted between the myocardial fibers o f 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 c onnection. The second patient presented with unsustained ventricular t achycardia and obstructive subaortic rhabdomyoma, requiring emergency surgery. One week later, reentrant supraventricular tachycardia develo ped and she required digoxin therapy for 15 months. Conclusions.-Infan ts with rhabdomyomatous tumor of the heart and ventricular preexcitati on syndrome may have microscopic or macroscopic accessory connections. Cardiac tumors like rhabdomyoma and oncocytic tumors should be suspec ted in infants with ventricular preexcitation syndrome or supraventric ular tachycardia.