Description of repair

Extracardiac conduit connects IVC to PA

Fenestration may or may not be used to serve as a “pop-off” valve to minimize central venous pressure and allow cardiac output in setting of increased mean pulmonary artery pressure.

Stage 3 Fontan Operation

Post operative management directed towards optimizing cardiac output at the lowest central venous pressure possible.

Post operative issues

Mechanical ventilation

Positive pressure ventilation may adversely affect PVR and ventricular filling.  Early extubation important.

Low cardiac output

May be caused by 1) inadequate preload, typically due to  hypovolemia, 2) elevated PVR, 3) anatomic obstruction in  the systemic venous pathway, or 4) pump failure.

 *Low cardiac output in face of high LA pressure is bad sign!

Arrhythmias

Poorly tolerated, especially loss of AV synchrony.

Commonly junctional ectopic tachycardia (JET), sinus node dysfunction, or atrial flutter.

Pacing may be needed!

Cyanosis – as previously described in stages 1and 2

Effusions/ascites

Typically most frequent postop problem prolonging hospitalization. Fenestrations and modified ultrafiltration have been helpful in reducing effusions.

Drainage catheters and appropriate replacement of volume, electrolytes, protein, and immunoglobulins is key.  

Thrombosis

Following surgery, at risk for venous thrombosis and central nervous system complications.

Anticoagulation is important.