CARDIAC FIBROMA

Course overview

CARDIAC FIBROMAS ARE THE SECOND MOST COMMON PRIMARY CARDIAC TUMOR AFTER RHABDOMYOMA IN CHILDREN. THEY ARE PRIMARILY COMPOSED OF CONNECTIVE TISSUE AND FIBROBLAST AND ALTHOUGH BENIGN, CAN BE SYMPTOMATIC DUE TO INTRACAVITARY OBSTRUCTION, INFLOW AND OUTFLOW TRACT OBSTRUCTION, CORONARY ARTERY COMPROMISE, THROMBOEMBOLIC EVENTS, CONDUCTION DEFECTS, AND CAN ALSO LEAD TO SUDDEN DEATH. PEDIATRIC CARDIAC TUMORS CARRY ASSOCIATIONS WITH GENETIC CONDITIONS, FOR EXAMPLE, RHABDOMYOMAS HAVE AN ASSOCIATION WITH TUBEROUS SCLEROSIS, AND ABOUT 3% OF PATIENTS WITH NEVOID BASAL CELL CARCINOMA SYNDROME (NBCCS), ALSO CALLED GORLIN SYNDROME, HAVE CARDIAC FIBROMAS. THIS ACTIVITY REVIEWS THE WORKUP OF CARDIAC FIBROMA AND DESCRIBES THE ROLE OF HEALTH PROFESSIONALS WORKING TOGETHER TO MANAGING THIS CONDITION.