Heart and heart-lung transplantation in Down's syndrome

The lack of supportive evidence means each case must be carefully assessed

Congenital heart disease is common in Down's syndrome, occurring in about 40% of individuals.1 Twenty years ago cardiac surgery was often not attempted in children with Down's syndrome because of operative mortality of up to 60% and a short life expectancy.2 With improvements in paediatric cardiac surgery and changes in attitude towards children with Down's syndrome such children now undergo corrective cardiac surgery. Some will inevitably develop complications and may benefit from heart transplant. There is also a large group of young adults with Down's syndrome who did not have heart surgery when young and who have uncorrected heart lesions that are now inoperable because of irreversible pulmonary vascular disease. They too are potential candidates for heart-lung transplantation. There is no published literature on heart or heart-lung transplantation in Down's syndrome, which makes it hard to predict the outcome in these patients.

Heart transplantation is now a widely accepted treatment, and medium term survival has steadily improved.3 The results of heart-lung transplantation are not as good but have …