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Purpose of the Review This is a comprehensive update on failing Fontan physiology and the role of heart and combined heart and liver transplantation in the current era. Recent Findings Single ventricle physiology encompasses a series of rare congenital cardiac abnormalities that are characterized by absence of or hypoplasia of one ventricle. This effectively results in a single ventricular pumping chamber. These abnormalities are rarely compatible with long-term survival if left without surgical palliation in the first few years of life. Surgical treatment of single ventricle physiology has evolved over the past 60 years and is characterized by numerous creative innovations. These include the development of arteriopulmonary shunts, the evolution of partial cavopulmonary connections, and the eventual development of the “Fontan” operation. Regardless of the type of Fontan modification, the long-term consequences of the Fontan operation are predominantly related to chronic central venous hypertension and the multi-organ consequences thereof. Atrial arrhythmias can further compromise this circulation.Patients with single ventricle physiology represent a special sub-segment of congenital cardiac transplants and are arguably the most challenging patients considered for transplantation. Summary This review describes in detail the challenges and opportunities of heart and liver transplantation in Fontan patients, as viewed and managed by the experienced team at the Ahmanson/UCLA Adult Congenital Heart Center.

Introduction This study aims to determine the diagnostic accuracy of Primovist-enhanced MRI in the detection of hepatocellular carcinoma (HCC) in cirrhosis, using liver explant histopathology correlation. Methods Thirty patients underwent a Primovist-enhanced MRI prior to liver transplantation for cirrhosis at the Princess Alexandra Hospital from 2006 to June 2012. Pre-contrast T1, T2 and post-Primovist arterial, portal venous and delayed hepatobiliary phase sequences were routinely obtained. A retrospective audit of MRI reports determined whether HCCs was identified on pre-transplantation imaging. Results were correlated to the explant liver histopathology. Specificity and sensitivity of Primovist in HCC identification in cirrhosis was determined. Results The time interval between preoperative MRI and transplantation ranged from the day of transplantation to 112 weeks pre-transplantation. Thirty-nine HCCs were identified histologically in explants. Fifteen HCCs were identified on pre-transplant MRI in 11 patients, ranging from 11 to 43 mm, all confirmed on histopathology. Fourteen MRI studies identified no lesion, concordant on histology. Twenty-four either well or moderately differentiated HCCs were identified on histopathology, all of which were less than 17 mm, were not depicted on MRI. No poorly differentiated HCCs were present in the explants. Two regenerative nodules were correctly identified on MRI. This correlated to a sensitivity of 42.5%, specificity of 100% and a positive predictive value of 100% and negative predictive value of 39.5%. Conclusion Primovist-enhanced MRI is very accurate in the identification of HCC in cirrhosis, especially if the tumour is greater than 17 mm and undifferentiated. However, sensitivity is reduced for well- and moderately differentiated HCC less than 17 mm.