There are approximately 1 million adult patients with congenital heart disease (CHD) in the United States, and the number is increasing. Hepatic complications are common and may occur secondary to persistent chronic passive venous congestion or decreased cardiac output resulting from the underlying cardiac disease or as a result of palliative cardiac surgery; transfusion or drug‐related hepatitis may also occur. The unique physiology of Fontan circulation is particularly prone to the development of hepatic complications and is, in part, related to the duration of the Fontan procedure. Liver biochemical test abnormalities may be related to cardiac failure, resulting from intrinsic liver disease, secondary to palliative interventions, or drug related. Complications of portal hypertension and, rarely, hepatocellular carcinoma (HCC) may also occur. Abnormalities such as hypervascular nodules are often observed; in the presence of cirrhosis, surveillance for HCC is necessary. Judicious perioperative support is required when cardiac surgery is performed in patients with advanced hepatic disease. Traditional models for liver disease staging may not fully capture the severity of disease in patients with CHD. The effectiveness or safety of isolated liver transplantation in patients with significant CHD is limited in adults; combined heart‐liver transplantation may be required in those with decompensated liver disease or HCC, but experience is limited in the presence of significant CHD. The long‐term sequelae of many reparative cardiac surgical procedures are not yet fully realized; understanding the unique and diverse hepatic associations and the role for early cardiac transplantation in this population is critical. Because this population continues to grow and age, consideration should be given to developing consensus guidelines for a multidisciplinary approach to optimize management of this vulnerable population. (HEPATOLOGY 2012;56:1160–1169)

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Perioperative myocardial infarction is an important complication of ligation of coronary artery fistulas and can contribute to reduced late survival. The tricuspid valve should be evaluated carefully at repair because of the relatively high rate of residual regurgitation in survivors.

Peripartum cardiomyopathy is a rare condition of unclear etiology that accounts for an important percentage of pregnancy-related deaths. Deaths from peripartum cardiomyopathy can be attributed to profound left ventricular failure, thromboembolic events, or arrhythmia. Prompt recognition of the condition, initiation of appropriate medical management, collaboration with perinatology for delivery management, referral to cardiac transplant centers when necessary, and counseling regarding future pregnancies is required for a successful outcome. Patients should be diagnosed by clinical evaluation and echocardiography. After establishing left ventricular dysfunction, a standard heart failure medical regimen should be instituted. Hospitalization should be considered for patients with class III or greater symptoms, or for those patients not responding to outpatient management. If the diagnosis is made in the antepartum period, delivery should be strongly considered. Endomyocardial biopsy has low yield in this situation and should not be considered standard care, especially because controversy exists over the effectiveness of immunosuppressive therapy for myocarditis. Selenium, pentoxifylline, and immune globulin have all been shown to have a beneficial effect in small series of patients. The addition of these agents to standard therapy, however, should be considered on a case-by-case basis. Anticoagulation should be considered in patients with ejection fractions less than 35%. Transplantation results in survival comparable to women with idiopathic-dilated cardiomyopathy, and should be pursued in the appropriate setting. Future pregnancies should be discouraged, even if the left ventricular function recovers. Significant improvement in ventricular function can be expected in up to 50% of patients.