LIFE-THREATENING CARDIAC INVOLVEMENT THROUGHOUT LIFE IN A CASE OF COSTELLO SYNDROME

Costello syndrome is characterized by poor postnatal growth, mental re tardation, curly hair, coarse face, loose skin of the hands and feet, and nasal papillomata. Patients with Costello syndrome have a high inc idence of cardiac involvement, such as arrhythmias, hypertrophic cardi omyopathy, or congenital anomalies. The importance of cardiac involvem ent in Costello syndrome has not been strongly emphasized thus far, al though arrhythmia and hypertrophic cardiomyopathy are both serious for ms of cardiac involvement. We report the case of a Japanese girl with Costello syndrome, who experienced life-threatening cardiac involvemen t throughout her life.