Of 207 pediatric patients who underwent orthotopic
heart transplantation at Loma Linda University Medical Center through January 1993, 96% of infants under 6 months of age and 45% of older infants and children had complex structural
heart disease. Twelve patients had
visceral heterotaxia, 10 had isolated
dextrocardia, and 16 had unusual atrial situs, including five with
situs inversus. Therefore a prerequisite for successful
heart transplantation in early life is a surgical team skilled in the management of
congenital heart disease.
Transplantation in very early life is usually primary
therapy, whereas in older infants and children it is an exercise in reoperative and reconstructive heart surgery. The keys to operative success include the following: (1) Appropriate recipient selection; (2) en bloc recovery of all veins and arteries required for anatomic reconstruction; (3) adequate myocardial protection; and (4) use of low-flow hypothermic perfusion and intermittent circulatory arrest as required. Although there is wide variability in the anatomic presentation of many potential recipients with
congenital heart disease, only two absolute anatomic
contraindications have been identified. Absence or marked hypoplasia of central pulmonary arteries and absence or markedly diminutive pulmonary veins are two anatomic variants that do not yield to
heart transplantation therapy. Previous
palliative surgery, variations of atrial situs, anomalies of pulmonary or systemic venous connections, and great vessel malformations all create challenge for the surgical team and potential risk for the recipient, but none preclude operative success.