The following are 10 points to remember about this scientific statement from the American Heart Association on transplantation and mechanical circulatory support in congenital heart disease:

1. A single-center study from 2003 including 26 patients showed varying outcomes based on the stage of palliation at the time of transplantation. The lowest mortality from transplantation for pediatric single-ventricle patients occurs within the bidirectional cavopulmonary connection stage. The 30-day survival for this group has been reported at 100% as compared to 62% for the systemic-pulmonary shunt group and 33.3% for the group with Fontan failure.
2. Exercise testing can provide prognostic information in patients with adult congenital heart disease (ACHD). A combination of peak VO₂ and heart rate reserve appears to provide the greatest predictive power. Expected normal values for exercise parameters vary based on the underlying congenital heart lesion.
3. ACHD patients have a high rate of sensitization because of previous surgeries requiring blood products and the use of homograft tissue. Panel reactive antibody (PRA) >25% prior to transplantation has been associated with increased mortality after transplantation.
4. Pulmonary hypertension is common in patients with congenital heart disease. ACHD patients with pulmonary vascular resistance (PVR) exceeding 4 Wood units have greater perioperative mortality risk (20%), although there is no difference in long-term survival based on pretransplant PVR.
5. Complex cardiac anatomy increases ischemic time and overall risk at the time of cardiac transplantation. Additional surgery at the time of transplantation is often required, particularly pulmonary artery (PA) reconstruction. PA reconstruction is required in 85% of Fontan patients and 42.9% of patients with other congenital lesions and is associated with increased mortality risk (odds ratio, 3.3).
6. Liver disease is a significant comorbidity in patients with congenital heart disease. This may be due to increased systemic venous pressure in Fontan patients, “right-sided” ventricular dysfunction and valve disease, and increased prevalence of hepatitis C. Hepatitis C has been reported in 8.6% of patients with ACHD who underwent surgery prior to routine screening of blood products in 1992.
7. Fontan circulation is associated with poorer outcomes after transplantation than other congenital lesions, with an 8.6-fold increase in relative risk of death. Patients with Fontan circulation and reduced ventricular function have a more favorable course than those with normal ventricular function and “failing Fontan” physiology. Some single-center studies have shown excellent transplant for Fontan patients. Patient selection and timing likely play a key role in determining outcomes.
8. Heart-lung transplantation may be considered for patients with Eisenmenger syndrome. The predicted survival of the patient with medical therapy (specifically pulmonary vasodilators) should be weighed against the survival rate after heart-lung transplantation (<60% 5-year survival).
9. Early survival after heart transplantation is lower in patients with ACHD than those with other pretransplantation conditions. However, late-term survival is better such that the median life expectancy (13 years) is better for ACHD patients than for all other pretransplantation diagnoses.
10. Mechanical circulatory support may be underutilized in the ACHD population. Complex anatomy impacts surgical planning for ventricular assist device placement in patients with congenital heart disease. Specifically, for patients with transposition of the arteries following atrial switch procedures, the systemic right ventricle is associated with inflow cannula occlusion and muscle resection is often required prior to inflow cannula placement.

Keywords: Cardiac Surgical Procedures, Eisenmenger Complex, Heart Defects, Congenital, Exercise Test, Fontan Procedure, Heart Transplantation, Heart-Assist Devices, Heart Failure, Heart-Lung Transplantation, Hepatitis C, Hypertension, Pulmonary, Liver Diseases, Survival Rate, Vascular Resistance, Vasodilator Agents, Ventricular Dysfunction, Ventricular Function

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