In patients with Fontan palliation, the systemic and pulmonary circulation is in series and supported by a single ventricle, resulting in fragile hemodynamics. Cardiac output is driven by low pressure nonpulsatile pulmonary flow and is highly dependent on pulmonary vascular resistance. An acute respiratory distress syndrome (ARDS) can rapidly alter this physiology and lead to severe cardiogenic shock. Herein, we describe the case of a 40 year old man with a classic modified Fontan procedure and bidirectional Glenn shunt who developed ARDS with cardiogenic shock following a resuscitated cardiac arrest with presumed aspiration pneumonia. In light of poorly tolerated positive end-expiratory pressure ventilation and underlying anatomical complexities, a multidisciplinary team was convened to optimize care. In part owing to the lack of femoral venous access, a veno-venous extracorporeal membrane oxygenation circuit was devised using bilateral internal jugular venous access. Under fluoroscopic guidance in a hybrid operating room, one cannula was placed in the inferior vena cava by means of the right internal jugular venous access, with the second cannula positioned in the right pulmonary artery through the left internal jugular vein. Oxygenation and hemodynamic status promptly improved, allowing the patient to recover from ARDS.