CARDIAC TRANSPLANTATION FOR END-STAGE CONGENITAL HEART-DEFECTS - THE MAYO-CLINIC EXPERIENCE

Objective: To review the outcome of cardiac transplantation undertaken in patients with congenital heart defects. Material and Methods: Betw een November 1991 and March 1998 at our institution, cardiac transplan tation was performed in 16 patients with congenital heart disease (age range, 3 to 57 years; mean, 26.1), Preoperative diagnoses included un iventricular heart (N = 4); complete transposition of the great arteri es (N = 3); Ebstein's anomaly (N = 2); tetralogy of Fallot (N = 2); le votransposition (N = 2); dextrocardia, corrected transposition, ventri cular and atrial septal defects, and pulmonary stenosis (N = 1); doubl e-outlet right ventricle (N = 1); and hypertrophic obstructive cardiom yopathy, (N = 1). All patients had undergone from one to five previous palliative operations. Results: Four patients required permanent pace maker implantation during the first month postoperatively because of b radycardia; more than 2 years later, another patient required a perman ent pacemaker because of sick sinus syndrome. In addition, one patient had an automatic implantable cardioverter-defibrillator. Three patien ts required reconstruction of cardiovascular structures with use of pr osthetic material (Teflon patches or donor tissue) at the time of card iac transplantation. Actuarial 1-, 2-, and 5-year survival was 86.2 +/ - 9.1%. During the first year after transplantation, two deaths occurr ed-one at 41 days of putative vascular rejection and the second at 60 days of severe cellular rejection. All other patients are alive and fu nctionally rehabilitated; the mean follow-up period has been 26.1 mont hs (range, 2 to 89.6). Conclusion: Cardiac transplantation for patient s with congenital heart disease can be accomplished with a low periope rative mortality and an excellent medium-Perm survival despite the cha llenges presented by the technical difficulties during invasive diagno stic procedures and at operation and the need for adherence to long-te rm multiple-drug therapy in this patient population.