With improved outcomes following palliative and corrective congenital cardiac surgery,
cardiac transplantation has decreased in recent years as a primary
therapy. Nevertheless,
congenital heart disease remains the most common indication for
cardiac transplantation during infancy. Primary
transplantation in infancy is selectively recommended for severe systemic
ventricular dysfunction, severe atrioventricular valve insufficiency, and occlusive coronary artery anomalies, particularly with single ventricle physiology. Wait-list mortality remains highest for infants with prior
palliative surgery and patients with failing Fontan physiology, both of whom have limited options for effective mechanical circulatory support. The sensitized patient carries an increased risk with prolonged wait times, although virtual cross-matches and single bead assays for donor-specific
antigens have facilitated the transplant process. Early and late survival after
transplantation for
congenital heart disease remain inferior to
cardiomyopathy, with prior
Fontan procedure as a major risk factor. However, among survivors at 6 months, late outcomes are generally excellent. Major late causes of death include allograft vasculopathy, post-transplant lymphoproliferative disease, and acute rejection. Noncompliance with medications remains a major risk for teenage mortality.
SUMMARY: