Combined En-Bloc Heart Liver Transplantation in Children with Congenital Heart Disease Complicated by Cardiac Cirrhosis

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Abstract

Introduction

Children with congenital heart disease now survive well into adolescence and adulthood. As palliative procedures reach the end of their lifespan, chronic conditions related to heart failure such as cardiac cirrhosis are becoming more prevalent. Combined heart-liver transplantation is an uncommon procedure offered to patients with heart and liver failure.

Materials and Methods

Seven patients underwent combined heart and liver transplant between the years 2006 and 2017 at a single institution. Indications, technique, and outcomes were reviewed. Donor organs were procured en bloc and placed into the recipient in similar fashion. Immunosuppression consisted of a standard protocol utilizing anti-thymocyte globulin, tacrolimus, mycophenolate mofetil, and steroids. Allograft ischemia, bypass, and total operative time, as well as blood product use, complications and length of stay were reviewed

Results and Discussion

Age at the time of transplant ranged from 8 to 23 years with a mean age of 17 years. Five patients had single ventricle physiology requiring Fontan procedures prior to transplant. Of the two remaining patients, one patient was diagnosed with teratology of Fallot and the other had double outlet right ventricle without a Fontan. All patients had findings of cirrhosis, portal hypertension and esophagogastric varices on pre-operative imaging and/or endoscopy. All patients had cirrhosis on their explant pathology. Graft and patient survival remains 100% with a mean follow-up of 5.2 years (8-4109d). There have been no episodes of rejection in either the cardiac or liver graft. One patient developed positive donor-specific antibodies (DSA) less than 10 days after transplant, which responded to IVIG and plasmapheresis.

Conclusion

Combined en bloc heart-liver transplant is a safe and effective procedure for those with end stage heart and liver disease. Advantages of the en bloc procedure include shorter ischemic times and brisk recovery of the transplanted organs. Patient selection relies on pre transplant biopsy and imaging in symptomatic patients. No long term or chronic rejection has been found as far as 10 years out from surgery suggesting an immune protective role of the liver.

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