En Bloc Heart and Liver Transplant for the Failing Fontan

History

The patient was a 23-year-old woman, born with single ventricle physiology (pulmonary atresia, hypoplastic tricuspid valve and hypoplastic right ventricle). She presented to the authors’ hospital with end stage heart and liver failure due to failing Fontan circulation. Her medical and surgical history was complicated. She had a history of multiple gastrointestinal (GI) bleeds, severe edema, protein losing enteropathy, and ascites due to liver failure.

Overall, she had undergone five surgical procedures, with the first one performed in 1994. The Fontan operation was completed in 1996, but subsequent stenosis in the Fontan circulation required two separate stent placements, one in the right pulmonary artery and the second one in the inferior vena cava (IVC)-Fontan anastomosis. On physical examination, the patient was small and frail, with ascites and +4 lower extremity edema. Her preoperative echocardiogram revealed normal left ventricular function, while liver evaluation revealed severe fibrosis and parenchymal disease. On the preoperative catheterization images shown in the video, one can appreciate the unobstructed flow through the Fontan circulation and the presence of two stents, one in the right pulmonary artery and the other one in the Fontan-IVC anastomosis. Multiple disciplinary discussions were held, and the decision was made to proceed with en bloc heart and liver transplant.

Surgery

The axillary artery was cannulated with an 8 mm side graft for arterial inflow, and bicaval cannulation was performed with a long 24 Fr cannula inserted percutaneously through the right femoral vein. Redo sternotomy was performed, and severe mediastinal adhesions were encountered. The abdominal transplant team started mobilizing the liver simultaneously by taking down ligaments and the adhesions. The IVC was circumferentially dissected and mobilized. As soon as the donor heart arrived, the superior vena cava (SVC) was cannulated and the patient was placed on cardiopulmonary bypass. The recipient heart was arrested with cardioplegia and removed.

Due to the Fontan circulation, there were a lot of collaterals and back bleeding. The previously placed stent in the right pulmonary artery was identified. Orientation was appropriate, and the left atrial anastomosis was performed first in a regular fashion. Meanwhile, the abdominal transplant team performed the abdominal vascular anastomosis sequentially, starting with the IVC, the portal vein, and the hepatic artery, followed by the bile duct. On the chest side, the most challenging part was the pulmonary artery (PA) anastomosis and reconstruction. The previously placed PA stent was fused within the wall and it was difficult to remove. The stent was trimmed to create free edges for the anastomosis. The posterior wall of the PA anastomosis was performed first, and donor pericardium was used for the anterior wall reconstruction, which was necessary due to a size discrepancy. The aortic anastomosis and the SVC anastomosis were performed in a regular fashion. Hemostasis was achieved, and the chest and abdomen were closed. The total cardiopulmonary bypass time was 206 minutes, cold ischemia time was 358 minutes, and circulatory arrest time was nine minutes.

The patient’s postoperative course was prolonged. She had one episode of GI bleeding that prompted a repeat endoscopy. Endoscopy revealed resolution of GI ectasias. The patient was discharged home on postoperative day 24, and she was doing well at her first follow-up appointment.

This was an extremely challenging case with an outstanding outcome, and the authors are grateful for the efforts of all the people involved with this patient’s care.