Pediatric Heart Transplantation

The results of cardiac transplantation in young children with complex congenital heart disease not amenable to surgical correction, or with end-stage heart failure due to cardiomyopathy, have shown significant improvement over the last decade. In view of the 60 percent 5-year survival and the 50 percent 10-year survival that patients can — on average — expect today, their good functional rehabilitation and enhanced quality of life, it seems justified that the option of pediatric heart transplantation should now be considered in the Netherlands. In order to realistically weigh the available therapeutic options, closely involving the parents, one should however point out the dilemmas that may confront all parties involved in pediatric transplantation. This concerns in particular the uncertain prospects of these children in the long run (the risk of chronic rejection, a possible need for re-transplantation). For the moment, it is fair to conclude that pediatric heart transplantation is an acceptable palliative therapy with good short and intermediate term results.

For the management of newborn infants with hypoplastic left heart syndrome (HLHS - a condition associated with an underdeveloped left ventricle, mitral valve and aortic root) the so-called Norwood staged repair operation offers an alternative to transplantation. The outcome of this palliative surgical procedure in the short and intermediate term, is comparable to that of cardiac transplantation. The availability of these options means that terminal care is no longer the only choice for these children.

A major challenge to all pediatric heart transplant programmes, is the present-day serious shortage of suitable donororgans. One can observe a huge discrepancy between the expected number of young transplant candidates in the Netherlands (estimated to be 25 per year at the most, including infants with HLHS), and the actual supply of suitable donor hearts in the wider Eurotransplant region (only around 45 per year, including 5 in the Netherlands). This shortage could well lead to unacceptably long waiting times and high mortality among patients on the waiting list. To avoid this situation, many centres nowadays show a preference for performing the Norwood procedure in infants with HLHS (even if transplantation is sometimes considered the superior option).

In view of the problems outlined above, the decision to start a pediatric heart transplant programme in the Netherlands should mean that patients with end-stage cardiac failure or with complex congenital malformations not amenable to conventional surgical repair or palliation, get first priority. This implies that available donor hearts should be allocated first to children with intractable cardiomyopathy or to infants with congenital defects in whom previous palliative surgery has not resulted in acceptable and durable improvement. In view of the present-day donor shortage, one should continue to offer the Norwood procedure as an option to newborns with HLHS (whenever life-prolonging medical intervention is deemed appropriate in such a child). As a result of this strategy, the expected number of transplants in the Netherlands will probably not exceed 10 per year during the first years of the programme.

Because of the expected small number of transplant candidates it seems reasonable to start with just one centre in the Netherlands. This situation will favor the development of the necessary expertise and will be beneficial to the quality and outcome of the transplants (despite a possible learning curve). Appropriate candidates are those university pediatric centres with proven expertise in pediatric cardiosurgery and organ transplantation. The candidate centre should also be in the position to profit from experience gained in an adult cardiac transplant programme. Again for reasons of quality, Norwood procedures should preferably be performed in a limited number of Dutch cardiosurgery centres (two or three at the most).

It is essential that a Dutch centre for pediatric heart transplantation should engage in further research concerning the long-term results of transplantation (survival, quality of life). Close co-operation with a well-established foreign transplant centre is also desirable.