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Key Concepts

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  • Epidemiology

    • Since 1982, over 8000 pediatric heart transplants have been recorded in the Registry of the International Society of Heart and Lung Transplantation. The current number of reported pediatric heart transplants is about 450 per year, which originate in 80 global centers.

  • Indications

    • Heart transplantation is the most effective and often the only treatment for end-stage pediatric heart disease. Complex congenital heart disease is the main indication for heart transplantation in infants, whereas end-stage cardiomyopathy is the main indication after the first year of life.

  • Contraindications

    • The main contraindication is elevated pulmonary vascular resistance that is unresponsive to oxygen and vasodilators.

  • Techniques

    • Heart transplantation is performed using either biatrial or bicaval techniques, depending on the size of the recipient child. Graft procurement and implantation are modified to accommodate the specific anatomic variations present in recipients with congenital heart disease.

  • Outcomes

    • Mortality while waiting for a donor is 12 to 40 percent. The overall actuarial survival at 10 years is 66 percent. It is 74 percent for newborn recipients. Causes of mortality include graft rejection and failure, infection, cardiac allograft vasculopathy (CAV) (16 percent), and malignancy (8 percent).

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Background

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The quest for clinical cardiac transplantation (CT) originated in research laboratories of the 1960s. Principal North American investigators included Norman Shumway and Richard Lower at Stanford University in California and Adrian Kantrowitz at Albert Einstein College of Medicine in New York, among others. While Shumway and Lower were focused on the potential for CT among adults, Kantrowitz’ studies were aimed at young infant recipients. At Stanford, investigators were learning the difficult balance between control of the host immune response and lethal opportunistic infections. Utilizing immunosuppressants of that era, adult laboratory animals were beginning to achieve survival of more than a year following orthotopic CT. In New York City, Kantrowitz observed that transplanted puppies experienced prolonged survival, even without immunosuppression. Then, unexpectedly, came news from Capetown, South Africa, that Christiaan Barnard and his team had accomplished the first clinical human CT.1 On December 3, 1967, 55-year-old Louis Washkansky underwent orthotopic CT. He died of pneumonia 18 days later, but the celebrated transplant stimulated global interest, and a bevy of similar transplants. The vast majority of these early recipients experienced abbreviated survival, dying of rejection, infection, or both.

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Just 3 days after the South African transplant, the first infant (neonatal) CT was attempted by Kantrowitz. The heart of an anencephalic infant was transplanted “off-pump” (i.e., with surface-induced hypothermia alone), into a neonate with severe Ebstein anomaly. The baby appeared to have a satisfactory operative procedure, but died suddenly and without clear explanation 6.5 h later. Kantrowitz did not pursue pediatric CT, and it would be 16 years before neonatal CT was to be attempted again. With the advent of cyclosporine for selective control of the cellular immune response,2 clinical pediatric CT was successfully performed in the summer of 1984. Eric Rose, of Columbia ...

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