Heart-Lung Transplantation in Young Children with CF

ABSTRACT & COMMENTARY

Synopsis: Although heart-lung transplants are generally reserved for older CF patients, children less than 10 years of age had similar three-year outcomes when compared to older patients.

Source: Balfour-Lynn IM, et al. Heart-lung transplantation for patients under ten with cystic fibrosis. Arch Dis Child 1997;76:638-640.

Lung or heart-lung transplantations are the final therapeutic option for end-stage lung disease in cystic fibrosis (CF). It is widely believed that transplantation is not an option for young children because of logistic problems in obtaining suitably sized organs and because previous reports have indicated that young children do less well than older ones.

Balfour-Lynn et al from the Transplantation Unit at Great Ormand Street Hospital for Children retrospectively reviewed 37 children who received transplants. Thirteen patients were under 10 years of age, and 24 were older. Three-year post-transplantation survival figures for these two age groups were similar (41% vs 46%). The authors conclude that pediatricians should not be deterred from referring younger patients for transplantation.

COMMENT BY THOMAS DOLAN, MD, FAAP

This is an interesting paper for historical reasons. This kind of study will probably never be repeated. I am struck by the large number of patients under 10 years of age that were assessed (58), placed on active transplant list (37), and given heart-lung transplants (13). Registry data from the United States CF Foundation for 1996 list only 31 deaths in children under 10 years of age from a total population of 19,456 CF patients. The mean survival for the first 10 years of life was 95.63%. The mean age of initial diagnosis has leveled in recent years at 2.8 years. With increasing use of neonatal screening tests, the age at diagnosis will undoubtedly continue to decrease. The survival of patients with CF has dramatically increased in the past 20 years so that the average survival is now approximately 30 years. This improvement is due to improved antibiotic regimes, nutritional therapy, and a team approach in management. Today it is very unusual to see a CF patient who is so seriously ill as to require hospital admission before 10 years of age.

The British have performed many heart-lung transplants. In North America since 1989, almost all transplants have been sequential bilateral lung transplantation procedures. Although the authors’ results with heart-lung transplantation are excellent, with 41% three-year survival, the main cause of death after a successful transplant is the development of bronchiolitis obliterans in the transplanted lung several years later. The etiology of this syndrome is incompletely understood but is probably a rejection phenomenon. The concern about bronchiolitis obliterans has deterred most American centers from performing lung transplants—especially in younger children.

The authors point out that there is a paucity of organs available for transplantation. Many patients succumb while awaiting a suitable donor. (Dr. Dolan is Professor of Pediatrics at Yale University School of Medicine.)