En bloc technique for Combined Heart-Liver Transplantation: A cumbersome strategy

Abstract

Background

Patients (pts) surviving after palliative Fontan-like surgery for univentricular heart are arising. Long-term hemodynamic changes often lead to Fontan-associated liver disease (FALD), which develops in almost all pts. These pts are at increased risk for hepatocellular carcinoma (HCC), or less commonly, for severe decompensated FALD requiring liver transplantation (LT). If required, sequential heart-liver transplant (CHLT) is the most common approach, however, according to scanty scientific data, en bloc technique offers surgical advantages, including shorter organ ischemic time and procedural simplification. We aim to describe the first two en bloc CHLT performed at our Center.

Methods

In 2024, in our Center two pts were listed for CHLT due to FALD and cardiac failure. We collected data about pre-LT management, surgical characteristics and post-transplant follow-up (f/u).

Results

Case 1: 38-years-old (yo) woman (blood group A) with congenitally corrected transposition of great arteries, left ventricular hypoplasia, pulmonary stenosis, sinus node dysfunction, right ventricle double outlet. Glenn and Fontan with extracardiac conduit surgeries were performed at age 1 and 7, respectively (total sternotomies 3). At the age of 17, epicardial pacemaker was implanted. At the age of 38, monofocal HCC was diagnosed (AFP 1042 ng/mL) and treated with stereotactic body radiotherapy (SABR), with partial response (CT scan after 3 and 6 months, AFP →82→14 ng/mL). 195 days (d) after SABR, she was listed for CHLT. She underwent en bloc CHLT 46 d after listing. Donor age 27-yo. Surgery lasted 12 hours (h), and 8 surgeons were involved; total ischemia time was 3.37 h. The cardiac portion of the procedure was carried out under cardiopulmonary bypass (CBP). Infrahepatic vena cava, common portal vein, hepatic artery, and common bile duct reconstruction included end-to-end anastomoses between graft and recipient.Transfusion requirement: blood units: 6, plasma: 600 ml. Pt was extubated on post-operative day (POD) 2 and weaned inotropes off by POD 6. The total length of hospital stay was 26 d. After 10 months she is alive, without HCC recurrence, and no signs of rejection on protocol biopsies. Case 2: a 34-yo man (blood group 0) with hypoplastic right ventricle and atrial septal defect (ASD). Glenn procedure and percutaneous ASD closure were performed at 9 and 15 years, respectively (total sternotomy 1). Cirrhosis was histologically diagnosed at 24-yo, later he experienced variceal bleeding, ascites and hepatic encephalopathy. He was listed for CHLT with NaMELD 20 and MELD XI 22 and after 274 d of listing, he underwent en bloc CHLT with NaMELD 24 and MELD XI 23. Donor age 28-yo. Surgery lasted 14 h and 8 surgeons were involved. CBP lasted 225 min and upgraded to ECMO due to acute right heart failure (+adrenaline, noradrenaline, iNO). Hepatic vascular and biliary reconstruction included end-to-end anastomoses between the graft and recipient’s infrahepatic vena cava, common portal vein, hepatic artery, and common bile duct. Transfusion requirement: blood units: 6, plasma: 1 L. ECMO was weaned off on POD 5. Hepatic graft function was satisfactory from the beginning, despite heart failure.Septic shock by Klebsiella Pneumoniae KPC occurred on POD 5. He underwent renal replacement therapy (from POD 7 to 21), tracheostomy (POD 13) and decannulation on POD 33. Post-transplant stay lasted 63 d (35 d in the intensive care unit). After a f/u of 8 months, the pt is alive with excellent function of both grafts.

Conclusion

As more pts with Fontan circulation reach adulthood, complications such as FALD are becoming increasingly prevalent. This trend underscores the growing need for expertise in CHLT, even though indications and surgical techniques are not yet standardized. In our experience, en bloc CHLT is a cumbersome but safe and effective strategy, decreasing operative times and allograft ischemic times, whereas offering protection of implanted allografts.