Heart transplantation is a recognised treatment for end-stage
heart failure of any cause including
congenital heart disease.
Congenital heart disease has contributed relatively little to the adult heart transplant activities in the past two decades. However, this is likely to change as an increasing number of children with
congenital heart disease reach adulthood because of the advances in paediatric cardiology and surgery. Some of these grown-ups with
congenital heart disease (GUCH patients) will need
transplantation for late myocardial dysfunction either secondary to uncorrected lesions, or despite previous repair or
palliative surgery. These patients are managed along the same clinical principles as those with
cardiac failure of other aetiologies, despite the lack of any evidence to support this approach. Nevertheless, they introduce new challenges. First, some may have pulmonary
vascular disease and require
heart-lung transplantation, or
lung transplantation combined with repair of their cardiac defects. Second, those with failing
Fontan circulation are usually much sicker than other transplant candidates, with
protein-losing enteropathy along with renal and hepatic dysfunction. Third, a suitable donor organ may not be found due to elevated levels of
antibodies in response to previous
blood transfusions and possibly the previous implantation of homografts. Fourth, the operation may be technically difficult because of the presence of adhesions secondary to previous operations, collaterals, and unusual anatomy. Fifth,
postoperative care may be complicated because of predisposition to
bleeding,
infection and
pulmonary hypertension, and the presence of residual aortopulmonary collaterals resulting in a significant left-to-right shunt. Despite a higher early mortality, the overall results of
heart transplantation so far have been encouraging with survivals similar to that of adults with acquired
heart disease and that of the paediatric population. However, this may change as the proportion of high-risk patients (failing Fontans) increases. GUCH patients with
Eisenmenger's syndrome may be offered
lung transplantation with repair of the cardiac defect or
heart-lung transplantation. However, because of the limited success of these approaches, and improved management of
pulmonary hypertension, patient selection remains difficult.