Single ventricle anomalies are rare congenital heart defects in which one of the ventricles (or chambers) of the heart does not develop properly.
The Fontan procedure is the last in a series of operations that tries to establish a near-normal circulation of blood for patients with functional single-ventricle physiology.
The goal of the procedure is to separate systemic and pulmonary blood flow by directing systemic venous return through the Fontan connection to the pulmonary arteries and the lungs without ventricular contribution.
Following the procedure, pulmonary blood flow is completely passive and dependent on pressure gradients, resulting in complex postoperative cardiopulmonary interactions.
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