Heart or Lung plus Liver - Professor Richard Kirk 2023

Multi-Organ Transplant
Introduction	Heart-Lung	Heart or Lung plus Kidney	Heart or Lung plus Liver

Heart or Lung plus Liver

The number of combined transplants is small - 12 heart-liver and 20 lung-liver in pediatric transplants between 1990 and 2017 as reported to the ISHLT registry (2018). Corresponding adult numbers are 272 & 133.

Indications

Liver disease may occur in as part of multi system diseases e.g. cystic fibrosis or .secondary to chronic congestion associated with cardiac failure - especially in the Fontan patient. Fontan Associated Liver Disease (FALD) is a progressive disease that leads to fibrosis, nodular formation, cirrhosis and ultimately hepatocellular carcinoma.

It is also recognized that some metabolic conditions require liver transplantation but also have severe impairment of cardiac function. For some patients a strategy of isolated liver transplant alone with ECMO support during the perioperative period may be beneficial compared to combined liver-heart transplant.

Assessment

In addition to meeting the criteria for the primary organ failure (heart +/- lung) criteria for liver transplant should also be met. A comprehensive assessment includes laboratory (GGT, bilirubin, Alk Phos, ALT, Platelets, INR), Endoscopy (varices), Ultrasound (fibrosis, cirrhosis), CT (HCC), MRI including Elastography (fibrosis, cirrhosis), Biopsy (not without risk) and MELD score (Modified MELD-XI - removes INR if on anticoagulation).

Emamaulle et al. Circulation. 2020;142:591–604

There is currently no good method for identifying the severity of FALD despite biomarkers, multiple imaging modalities and venous congestion or biopsy results. This makes establishing irreversibility criteria and therefore the decision for multi organ transplant difficult, but essential as combined (simultaneous) outcomes are better than transplanting the liver at a later date.

The Stanford pediatric and young adult experience suggests that combined transplantation has better outcomes once cirrhosis is evident (Sganga et al). One recent proposal in adult FALD from a multi-institutional collaboration by Lewis et al (see further reading) suggests a FALD score >2 based on scoring 1 for each of the presence of cirrhosis (either imaging or biopsy), varices, splenomegaly, a score >2 predicts better survival than cardiac transplant alone. However the Toronto experience suggests that in many cases, even with cirrhosis, favourable outcomes can occur without combined transplant (Broda et al).

Management

Rejection after combined Heart-Liver or Lung-Liver transplant is less compared to a single organ only. It is postulated that as the liver is transplanted first donor antigens are consumed in the liver. It is possible that induction strategies can therefore be modified. Likewise long term immunosuppression may be able to be reduced with beneficial effects on the adverse consequences of immunosuppression.