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Revision Fontan procedure in an adult with single ventricle physiology

At 23 years old, Jessica Johnson was attending her weekly art class when she suddenly lost all her vision and began to have difficulty breathing and speaking. Johnson was rushed to a local hospital, where she underwent testing.

Johnson was born with a double inlet left ventricle and underwent multiple surgical procedures to correct this. At age three, she underwent a “classic” atriopulmonary Fontan operation in which the right atrium was anastomosed directly to the pulmonary artery, essentially allowing her to live off of one ventricle in her heart rather than two. Her functional single ventricle only supported circulation to the body, and there was no ventricular chamber pumping blood to the lungs. Over the subsequent years following the Fontan procedure, her right atrium became massively dilated, leading to