Abstract
We present a rare case of giant rhabdomyoma of the right heart ventricle entire cavity with complete atrioventricular orifice and right ventricular outflow tract obturation in the newborn period and subsequent tumor reduction by adolescence with total septal and posterior tricuspid valve leaflets involvement. In the neonatal period, due to the right heart ventricle entire cavity obstruction with rhabdomyoma, a Blalock–Taussig–Thomas systemic-to-pulmonary shunt (BTTS) No. 3.5 was applied to the child on the eighth day of life. After hospital discharge, the patient was annually followed up by a cardiologist at Children’s City Hospital No. 1 in St. Petersburg during 5 years. In dynamics, the gradual regression of rhabdomyoma nodes with the 2nd degree tricuspid insufficiency development was observed. Later, the child fell out of monitoring and was admitted to the Cardiac Surgery Unit of the Children’s City Multidisciplinary Clinical Specialized Center for High Medical Technologies in 2024 at the age of 15. At the time of admission, echocardiography data identified 3-4th degree tricuspid valve insufficiency with two cusps reduction, atrial septal defect (ASD) up to 9.0 mm with right-to-left shunt. The tricuspid valve plastic surgery with cone reconstruction method and the ASD closing with a small residual flow were performed on a child.
