high Improvements in transplant strategy and earlier referral have improved results Using the donor branch pulmonary arteries greatly facilitates the transplantation Because infection causes the majority of early deaths in these patients, lower-intensity immunosuppression is warranted At the Lurie Children's Hospital, transplantation was successful in treating PLE in all survivors The 5-year survival in the early portion of that series (1990 to 2011, n = 18) was 60%.394 Since 2012 (n = 20) the 5-year survival has been 95% A very small number of failing Fontan patients may benefit from a ventricular assist device, and most recently there has been success reported with the SynCardia device (SynCardiac Systems), an implanted total artificial heart This continues to be an area of investigation in selected centers FIG 73.29 Kaplan-Meier survival curve of freedom of death and transplantation in the early conversion center versus the other centers (From Poh CL, Cochrane A, Galati JC, et al Ten-year outcomes of Fontan conversion in Australia and New Zealand demonstrate the superiority of a strategy of early conversion Eur J Cardiothorac Surg 2016;49[2]:530– 535.) Management of the patient with a “failing Fontan” requires a multidisciplinary evaluation by pediatric cardiologists, pediatric cardiovascular surgeons, electrophysiologists, interventional cardiologists, and transplant surgeons Optimizing outcomes is dependent on early referral for appropriate therapy at centers with the required depth of institutional experience to manage these complex patients Timing of Heart Transplantation In general terms, referral for heart transplantation should occur at a time when expected survival with transplant exceeds that without a transplant Some Fontan failure patients can remain stable with medical therapy for years without transplantation, whereas some experience a precipitous decline that is often difficult to predict Thus there is a fine line between being “too well” and “too sick” for transplantation that can be elusive in the Fontan patient Risk assessment for transplantation is much better understood in patients with normal cardiac anatomy and two ventricles compared with those with a functionally univentricular heart along the Fontan pathway It follows that the criteria for listing for heart transplantation are primarily based on the risk profiles that are more typical for patients with two ventricles with diminished ventricular function Listing criteria vary from country to country and are typically more stringent in adult patients with variable provisions for exceptional listing status These criteria typically involve objective measures of cardiac function, exercise capacity, hemodynamic instability, need for inotropic support, or need for mechanical support, with sicker patients receiving priority for organ allocation These listing criteria are particularly ill suited for adult Fontan patients, particularly as evidenced by significantly longer wait-list times and higher perioperative mortality.394,399,400 Survival after the perioperative period and after the first year following transplant is better in patients with CHD, but there are no specific long-term data for those with a previous Fontan circulation.400 Given the high prevalence of cirrhosis and other forms of Fontan-associated liver disease in these patients (see earlier), it is important to determine whether there is a need for combined heart-liver transplantation, which has a higher perioperative risk and is performed far less frequently than heart transplant Most patients who require combined heart and liver transplantation have systemic disease such as amyloidosis, familial hypercholesterolemia, or iron overload syndromes (hemochromatosis and thalassemia), and there is far less experience in the patient with a failing Fontan.401,402 Practices vary, and there is no agreed upon way of determining the need for liver transplantation in conjunction with heart transplantation in patients with a failing Fontan Although liver biopsy is the gold standard, its sensitivity is variable, and it may be of limited utility because of the patchy and heterogeneous nature of fibrosis—with denser fibrosis in the hepatic periphery.403,404 This may explain lack of correlation with clinical outcomes in the Fontan population In non-Fontan patients, the MELD score is a validated and widely used risk calculator that is used for assessing prognosis and listing priority for cirrhotic patients listed for liver transplantation MELD score calculation uses serum creatinine, bilirubin, INR, and serum sodium, the latter being added in 2016 The Pediatric End-stage Liver Disease (PELD) score is a similar to MELD and is used for pediatric patients younger than 12 years of age The PELD score uses albumin, bilirubin, INR, age, gender, height, and weight PLE is associated with lower bilirubin due to gastrointestinal loss of proteins that include albuminbound bilirubin, so these patients will have lower MELD and PELD scores In addition, the use of vitamin K antagonists can elevate the INR and increase MELD and PELD scores Liver imaging with MRI, CT, ultrasound and, more recently, transient elastography can help to quantify fibrosis This, in conjunction with clinical features of decreased renal perfusion and portal hypertension, such as proteinuria, varices, ascites, splenomegaly, and thrombocytopenia, can be of prognostic value and can help to determine whether liver transplantation is warranted in conjunction with heart transplantation Improving the assessment of prognosis in the failing Fontan is of critical importance when considering whether or not to refer for transplantation The MELD-XI score is a modification of the MELD score that excludes INR and sodium in the calculation.154 Higher MELD-XI scores predict death or transplantation, but this score tends to be a surrogate marker for decreased renal function because it is mostly driven by elevated creatinine Renal resistive index is a marker of renal perfusion and is increased with elevated CVP, wide pulse pressure, hypoxia, heart failure, liver dysfunction, and diuretic use A report demonstrated increased mortality in the Fontan patient with renal resistive index of 0.81 or greater.405 Others have reported a high VAS score (one point each for varices, ascites, and splenomegaly by imaging) in addition to cyanosis or the need for pacemaker to be associated with a higher incidence of death or need for transplant.406 An important observation was that a VAS score of 0 was associated with a very low 10-year event rate, which could help identify a group of patients who would not benefit from transplantation Although these risks scores or diagnostic methods may be useful tools for serial monitoring of the failing Fontan patient, none has been validated prospectively as a means of determining prognosis or timing of transplantation referral Further study is needed to help identify patients with a functionally univentricular heart Fontan who are at highest risk of 12- to 24-month mortality Identifying these patients may help to refine specific and appropriate transplant ... creatinine, bilirubin, INR, and serum sodium, the latter being added in 2016 The Pediatric End-stage Liver Disease (PELD) score is a similar to MELD and is used for pediatric patients younger than 12 years of age The PELD score uses