A 17 years old male with a extracardiac Fontan, plastic bronchitis, and evidence of cirrosis with one precancer nodule, is listed for heart transplantation.
Liver function is still good.
Do you think it's enough or is better to list him for combined heart-liver transplantation?
Hello Carlo
The answer will depend on the clinical and functional status of the patient...
As per the information you've given, a double transplant in a failing Fontan seems too much surgery with too little benefit. Maybe heart transplant and hepatic embolization...
Again, functional status is the most important variable for deciding in such a complex patient
Hi Helena,
the patient is suffering more for the plastic bronchitis than for the liver. Moreover, there is evidence at the MR of numerous spleno-renal shunts that seem to reduce the portal hypertension. I'm worried that the heart transplantation will be hard to survive to, but I think that the risk of the combined heart-liver transplantation is too high and the result at the long term follow-up will be the same. Analyzing the post Fontan hepatopaty with the same criteria of post hepatitis cirrhosis can be misleading.
Suggest you list for combined heart liver transplantation. The patient will not do well if you undertake isolated heart transplantation in the setting of cirrhosis and portal hypertension. The liver lesion can be addressed by tracking the patient with sequential imaging whilst they are on the waiting list. There is some published evidence to support this approach-
Article Isolated heart transplant and combined heart-liver transplan...
But this means a longer operation time, and a higher risk. Our hepatic surgeons are worried about the option of postoperative ECMO and the need to anticoagulate the patient. This option is quite real in my mind. I really don't know if it's worthwhile to undergo combine di transplantation.
Failing Fontan are a terrible subset of patients... and this patient has pulmonary disease to increase the risk.
It's a decision between the ideal best solution (which might be the combined transplant) and the best solution for this particular patient.
It's a very difficult decision...
I agree with you. The decision must suite to that particular patient, and it's difficult to be sure of what to choose. I'll let you know what will happen, thank you very much for sharing your experience.
Success comes from trying and understanding what is required for good outcomes -selection of good quality donor organs is a must and one of the other caveats is that the patient can be weaned from bypass etc for the liver transplant to proceed.
Experience with other combined procedures helps-eg lung-liver transplantation and cardiac bypass surgery combined with liver transplantation.
One other option is to refer the patient to a centre with expertise if that is possible. This is an increasing problem as more young patients survive into adulthood post cardiac surgery for congenital heart defects.
The question is what to do, if there is the indication to combined transplantation or not. Our transplant center is trained in pediatric transplantation and combined transplants, so the option can be delivered. What I mean is if combined transplant is justified be a precancer lesion in a failing Fontan liver.
The patient underwent heart transplantation on December 2017. Post-operative chilothorax was treated by thoracic duct ligation and low-fat diet. After a transitory acute kidney insufficiency, treated by CVVH, the renal function is now optimal. Colchicine was used to treat the tendency to anasarcatic state due to AKI and lymphatic stasis secondary to thoracic duct ligation and chronic venous ipertension due to Fontan circulation. No problems related to liver function were evidenced. The patient was discharged and we plan to check his liver in the next months. I'll let you know the results. Thanks to all for the suggestions.
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