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Background
The Fontan operation is a palliative procedure performed in patients with a functionally or anatomically single ventricle that is not suitable for a biventricular repair.
The venous blood is diverted directly to the pulmonary arteries usually either via the right atrium (direct right atrium-pulmonary artery Fontan) or via a tunnel (either a lateral tunnel Fontan or extracardiac conduit). There is generally no subpulmonic ventricle to provide pulsatile blood to the pulmonic circulation. Pulmonary blood flow is thus dependent on changes in intrathoracic pressure and passive flow from the systemic veins to the pulmonary bed.
Patients with the Fontan operation are at risk of various complications including”
1. Arrhythmias – re-entrant atrial arrhythmias are particularly problematic
2. Heart failure
3. Pulmonary and systemic thromboembolism.
4. Hypoxia from intracardiac and intrapulmonary communications
5. Protein-losing enteropathy
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