Fontan Conversion in a Patient With Hypoplastic Left Heart Syndrome: A 38-Year-Old Norwood Saga

A 38-year-old male with hypoplastic left heart syndrome underwent the initial three palliations, with a final intra-atrial conduit Fontan. He presented with desaturation and liver dysfunction, and it was noted that his Fontan conduit was quite small (12 mm) and calcified. Options included combined liver/heart transplantation or replacement of his Fontan conduit. Due to his satisfactory ventricular function and Fontan hemodynamics, the decision was made to proceed with replacement of the Fontan conduit. 
 
Through a fourth sternotomy, the innominate artery, both the superior vena cava, and the right common femoral vein were cannulated for cardiopulmonary bypass (CPB). After cardioplegic arrest, an atriotomy was performed, and the Fontan conduit was resected. The pulmonary confluence and the inferior vena cava were disconnected from the atrium, and both atrial ends were oversewn. A 20 mm polytetrafluoroethylene graft was then used for the extracardiac Fontan reconstruction. The patient was subsequently weaned off CPB without difficulty and extubated in the operating room.He was discharged seven days later. 
 
While uncommon, replacement of a small or obstructed Fontan conduit is a valid solution to improve hemodynamics and prevent further liver deterioration. This approach may be considered to delay the need for transplantation, especially if the Fontan hemodynamics remains satisfactory. 

References

1. Said SM, Mashadi A, Essa Y, Erb M. Fontan Conversion in an Adult With Hypoplastic Left Heart Syndrome: A 38-Year-Old Norwood Success. JACC Case Rep. 2024;29(17):102495. Published 2024 Sep 4. doi:10.1016/j.jaccas.2024.102495
2. Said SM, Burkhart HM, Dearani JA. The fontan connections: past, present, and future. World J Pediatr Congenit Heart Surg. 2012;3(2):171-182. doi:10.1177/2150135111434806

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