Pediatric heart-lung transplantation for cystic fibrosis.

OBJECTIVE

To describe the preoperative and postoperative experience of children who have undergone heart-lung transplantation for cystic fibrosis.

DESIGN

Retrospective descriptive study.

SETTING

Paediatric Surgical Unit, Harefield Hospital, Middlesex, Great Britain.

PATIENTS

Twelve children less than 15 years of age (mean age 11 years 10 months; range 7 to 14 years), six boys and six girls, who received heart-lung transplants between September 1987 and March 1991.

RESULTS

All 12 children were alive and well 1 year after surgery, although one girl had undergone retransplantation in the eighth postoperative month. Actuarial survival rate was 66% at 2 years.

CONCLUSIONS

Early results suggest that heart-lung transplantation is a successful therapeutic option for children with cystic fibrosis. Cystic fibrosis-related postoperative complications were malabsorption of immunosuppressive drugs, meconium ileus equivalent, and persisting infection in the upper respiratory tract. These and general complications of acute rejection and infection did not prevent 66% of the group from returning to their normal schooling within the first postoperative year. Obliterative bronchiolitis remains the most serious late complication after lung transplantation, and further research is needed into treatment and prevention.