Tricuspid Atresia

Abstract

Tricuspid atresia (TA) “is a rare cyanotic congenital heart defect, defined by total agenesis or absence of the tricuspid valve, resulting in an obstruction of the blood flow between the right atrium and ventricle.” Therefore, a right-to-left shunt at the atrial level is obligatory for survival, resulting in the mixing of the systematic and pulmonary venous circulation. The impaired oxygen saturation and the underlying pathophysiological changes in the left ventricle lead to the clinical manifestations of the disease that include cyanosis and cardiac failure in the early life. The severity of the clinical presentation depends upon the flow in the pulmonary circulation. Apart from the postnatal diagnosis in neonates or young infants, most recent advances in fetal echocardiography permit the accurate prenatal diagnosis of the TA. Without the appropriate treatment, TA is associated with high mortality rates. Several surgical procedures such as the corrective Fontan operation have altered the clinical course of this congenital defect. This resulted in an improvement of patients’ life expectancy and quality of life.