A cardiac rhabdomyoma is the most commonly occurring tumor found in the pediatric population. Rhabdomyomas may occur in multiple organs and are associated with Tuberous Sclerosis, a genetic, autosomal dominant syndrome. 50% of patients with Tuberous Sclerosis develop cardiac rhabdomyomas. Rhabdomyomas are considered benign abnormal growth of cardiac myocytes which may become problematic if their growth inhabits critical intracardiac locations, resulting in obstruction (inflow/outflow) and/or potentially lethal dysrhythmias. Historically, cardiac rhabdomyomas do not metastasize and in most cases, naturally regress over time. There may be complete or partial regression with a survival rate of 81-92%. Echocardiography is the preferred diagnostic tool used in the detection and evaluation of progression and regression of cardiac rhabdomyomas. Rhabdomyomas may be diagnosed pre or postnatally.  They appear as well defined, homogenous, hyperechoic masses which range in size and may be isolated or diffuse. Rhabdomyomas are most commonly found in the ventricular myocardium but may also be found in the atria, cavoatrial junction, and epicardial surface.


Echocardiographic Assessment:

Focused interrogation of right and left sided inflow and outflow tracts to assess for obstruction

Location, size and number of lesions