Hence, there is only limited experience

with transplanting persons with CHD and liver disease. In addition, the severity of cardiac dysfunction among the above-described cases is not known. Overall survival of patients receiving heart transplants in the United States for CHLT is 83% (3 months), 74% (1 year), and 64% (5 years), respectively. However, this excellent survival may be driven by the unique characteristics of the population. Most patients undergoing CHLT have amyloidosis, and these patients are often young to middle-aged with normal liver synthetic function and minimal find more coagulopathy.41 The risk of the procedure is often determined by the cardiac disease, rather than the liver disease. At our center, we have performed CHLT for 3 patients with complex CHD and cardiac cirrhosis (MELD range, 10-15) with 100% survival (range, 8 months-4 years). In patients with

failed Fontans who have had multiple transfusions, there is the risk of sensitization to donor antibodies, which makes receipt of a suitable organ challenging. The multiple sternotomies and cardiac procedures greatly increase the technical complexity of the cardiac transplant. Transplanting the liver before the heart may serve to absorb donor-specific antibodies, which can cause cardiac rejection, but places the liver at increased risk of ischemia in the absence of adequate cardiac function. In the 3 patients with CHD and cardiac cirrhosis undergoing CHLT, all of the patients were sensitized FK228 price to donor antibodies; though there were episodes of acute cellular rejection, there were no episodes of antibody-mediated rejection. Patients listed for CHLT often get transplanted based on their cardiac status,

rather than the MELD score. Wait-list mortality for the average candidates listed for the CHLT dual waiting list (cardiac status 2 and MELD scores of 20-29) approximates the waiting-list mortality of those with status 1 or a MELD score higher than 30.40 After CHLT, lower immunosuppression levels are tolerated with a lower risk of graft rejection related to induction of partial tolerance.41, 42 In 93% of patients undergoing CHLT at the Mayo Clinic, both surgeries were completed in a single stage without perioperative mortality.41 As compared MCE to a control group undergoing heart transplant alone, rejection rates were lower and pulmonary embolism was higher in the CHLT group, but survival was similar between the two groups. Significant strides have been made in reducing mortality in patients with CHD. However, the long-term sequelae of palliative procedures in early childhood are not yet fully realized, and an increase in morbidity attributed to liver disease, especially with the associated and potentially increased risk of HCC, is expected over the lifespan of this vulnerable population.