310 Aorto-pulmonary anastomosis in tuberous sclerosis and cardiac tumor with severe right ventricular outflow tract obstruction (a case report)

Abstract

Primary cardiac tumors are rare with an estimated incidence of 0.27% in pediatric autopsies. The most common type of cardiac tumor identified in infancy and childhood is rhabdomyoma. We report a case of prenatal diagnosis of multiples cardiac rhabdomyoma at 29 weeks of gestation. Full-term delivery was straightforward without hydrops and dysrhythmia. In the immediate post-natal period, cyanosis appeared, and echocardiography showed multiple cardiac rhabdomyoma and severe right ventricular outflow tract obstruction (Image 1). Aprostadil perfusion was necessary. Aorto-pulmonary anastomisis was performed with success. The diagnosis of cardiac rhabdomyoma was confirmed histologically and tuberous sclerosis by molecular genetic analysis. Renal echocardiography was normal and cerebral MRI was not perfomed. Fourteen months later, neurodevelopment was normal. Echocardiography confirmed regression of the cardiac tumors with disappearance of the severe right ventricular outflow tract obstruction. ECG monitoring was normal. The natural history of most cardiac rhabdomyoma is favorable with tumors regressing (completely or partially). Surgical resection of the tumors is required for severe ventricular outflow tract obstruction. Aorto-pulmonary anastomosis, as in our case with severe right ventricular outflow tract obstruction, is an alternative treatment because regression and even complete resolution of more than 80% of the tumors occurs during infancy and early childhood.