The Fontan operation is a palliative surgical procedure performed in patients with a functional or anatomic single ventricle (also known as univentricular heart). The Fontan operation and its modifications have remained an important milestone in the surgical management of selected patients with complex cyanotic congenital heart disease.
Hypoplastic left heart syndrome (HLHS)
Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect (CHD), it is spectrum of congenital cardiac defects characterized by “underdevelopment of the left heart significant hypoplasia of the left ventricle including atresia, stenosis, or hypoplasia of the aortic and/or mitral valves as well as the ascending aorta and aortic arch. occurring in 2 per 10 000 livebirths. It is fatal without surgical intervention and responsible for 25% to 40% of all neonatal cardiac mortality.
There are three main types of HLHS:
1)Aortic atresia with mitral atresia: most severe, retrograde blood flow, and systemic output is entirely PDA dependent.
2- Aortic atresia with patent mitral valve: inflow without outflow, resulting in LV hypertrophy. 3-Aortic valve stenosis with patent mitral valve: antegrade blood flow, mild end of the HLHS spectrum. …show more content…
Instead, there is reliance upon an energy gradient between the systemic veins and the pulmonary artery without a pressure gradient. Pre-operative factors affecting this gradient will be problematic during the post-operative course and must be clearly communicated to those participating in the patient's management. Those with elevated pulmonary artery pressures may not be acceptable candidates for the procedure as the systemic venous pressure may be unable to overcome the resistance and provide forward blood flow17. Thus, the Fontan is not performed in the neonatal period due to the presence of normally high pulmonary vascular