Abstract
Heart transplantation is an accepted therapeutic modality for end-stage congenital heart disease for both biventricular and univentricular anomalies. Many transplant centers have pushed the limits of transplantation to include patients with high pulmonary vascular resistance, high panel reactive antibodies, positive cross-matches, and ABO-incompatibility. Excellent results have been possible, particularly with the development of improved diagnostic and therapeutic algorithms to prevent and treat rejection, infection, and post-transplant lymphoproliferative disease. Late graft failure and chronic rejection remain vexing problems. The vast majority of patients with biventricular congenital heart disease have undergone prior cardiac surgical procedures. Indications for transplantation in this subgroup are primarily progressive refractory heart failure following prior cardiac surgical reconstructive procedures. Contraindications to transplantation mimic those for other forms of end-stage heart disease. A determination of pulmonary vascular resistance is important in listing patients with biventricular congenital heart disease for heart transplantation. Modifications in the implant technique are necessary and vary depending on underlying recipient anatomy. Risk factors for perioperative outcomes in patients with biventricular congenital heart disease include the need for reoperation, the degree of anatomic reconstruction necessary during the implant procedure, and the degree of antibody sensitization, in addition to a number of other recipient and donor factors. Postoperative outcomes and survival are very good but remain inferior to those with cardiomyopathy in most series. In conclusion, patients with end-stage biventricular congenital heart disease represent a complex group of patients for heart transplantation, and require careful evaluation and management to ensure optimal outcomes.
Highlights
The first pediatric heart transplant was performed in 1967 for an eighteen day old patient with congenital heart disease (CHD) consisting of Ebstein’s anomaly [1]
Biventricular CHD was present in 44 patients, with transposition representing the biggest cohort (D-the great arteries (TGA) in 10, L-TGA in 6), followed by right ventricular outflow tract lesions, Ebstein’s anomaly in 3, and other diagnoses in 13
The following list represents contraindications to transplantation, which applies to patients with biventricular CHD as well as any other patients undergoing consideration for heart transplantation:
Summary
The first pediatric heart transplant was performed in 1967 for an eighteen day old patient with congenital heart disease (CHD) consisting of Ebstein’s anomaly [1]. End-stage CHD of the biventricular form is a heterogeneous group of patients, almost all of whom have undergone prior surgical repair This monograph will highlight the indications, contraindications, recipient anatomic considerations, donor evaluation, surgical techniques, survival, and long-term outcomes following heart transplantation for biventricular CHD, both in adult and pediatric recipients. Cardiac catheterization should be performed in these two patient groups: those with native coronary artery stenoses should undergo consideration for primary heart transplantation as the mortality of surgical palliation is high. While this study did not stratify patients based on biventricular versus univentricular CHD, a number of comments can be made: Of the patients with biventricular CHD, TGA, either D- or L-TGA, accounted for the biggest subset of biventricular CHD patients (97 patients, 31%) This was due to systemic ventricular failure, often a systemic right ventricle.
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