SPONTANEOUS REGRESSION OF CARDIAC RHABDOMYOMA

Multiple cardiac rhabdomyomas in a neonate with tuberous sclerosis were first described by Von Recklinghausen in 1862. These hamartomas are the cardiac tumors most frequently encountered during infancy and childhood. Rhabdomyomas account for 45% of primary heart tumors in children and represent 53% of primary benign childhood cardiac tumors.1 Approximately 30% of patients with tuberous sclerosis have cardiac rhabdomyomas.2 Their natural history is unclear because most reviews on this subject are based on autopsy data. The prognosis for cardiac rhabdomyomas is believed to be grim because of reported fatality rates of 53% by the first week of life and 78% by 1 year of age.2,3 With widespread use of echocardiography in pediatrics during the last 2 decades, it has become clear that rhabdomyomas result in a wide spectrum of clinical manifestations, ranging from a total absence of symptoms to intrauterine or sudden postnatal death. Also reported are hydrops fetalis, dysrhythmias, inflow or outflow obstruction, congestive heart failure and possibly cerebral embolization. Histologic examination of these masses in 1923 was suggestive of spontaneous regression.4 Isolated clinical reports of spontaneous regression have recently appeared.5 We now describe a series of 5 infants with tuberous sclerosis who had close documentation of the size of their 13 tumors. © 1991.