Congenital heart defects (CHD) are among the common diseases in childhood. According to global statistics, the overall incidence of children born with these defects is 6-8 per 1000 live births. There are over 40 nosological forms of CHD, but the most prevalent are 9 forms that constitute approximately 85% of the total. The remaining 15% encompass rare and complex forms.A unique feature, encountered very rarely and constituting a nosological discovery in adult therapeutic practice, is Ebstein anomaly (EA)