The path to Fontan: Echocardiography of the single ventricle

Abstract

The Fontan procedure was developed in 1971 for the palliative repair of tricuspid atresia. This operation results in flow of systemic-venous blood directly to the lungs without passing through a ventricle. It has been adapted over the decades to treat several complex congenital heart abnormalities commonly grouped as single ventricles or more accurately, functional single ventricles. The main characteristic of this group is the under development of one ventricle which makes the heart incapable of supporting both the systemic and pulmonary circulation, ruling out a biventricular repair. Functional single ventricles can be divided into single left ventricles with a rudimentary right ventricle (tricuspid atresia, double inlet left ventricle, severe Ebstein's anomaly), single right ventricles with a rudimentary left ventricle (hypoplastic left heart syndrome, mitral atresia, complex double outlet right ventricle), a solitary and indeterminate ventricle and other heart conditions not amenable to a biventricular repair (unbalanced atrio ventricular septal defect (AVSD), multiple ventricular septal defects (VSDs), straddling atrioventricular valves). Echocardiography forms the main diagnostic investigation and ongoing management modality for those with a functional single ventricle condition. An accurate segmental, sequential echocardiographic assessment is essential for diagnosis and follow up for patients moving along the palliative Fontan path.L6165328852017-06-06 <br />