The Lymphatic System: The Achilles Heel of the Fontan-Kreutzer Circulation.

In spite of excellent long term survival the Fontan Kreutzer procedure commonly presents late failure due to end-organ damage. Several advances have been described to refine single ventricle management and surgical techniques. However, very little research has been dedicated to the lymphatic circulation in the precarious Fontan hemodynamic state. The lymphatic circulation is clearly affected since there is increased lymph production, which requires to be drained at a similar or higher pressure than it is produced, commonly resulting in chronic lymphedema. Chronic lymphedema induces fibrosis and end-organ failure even in normal circulation. Diverting lymph drainage to the low-pressured systemic atrium in Fontan may represent a valid alternative for the treatment of devastating complications as protein-losing enteropathy and plastic bronchitis and may prevent or decrease the development of end-organ fibrosis or failure.