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Heart-lung transplantation for cystic fibrosis. 2: Outcome
B Whitehead1, P Helms, M Goodwin
1Cardiothoracic Unit, Hospital for Sick Children, London.
Insights
Combined heart-lung transplants improved quality of life for children with cystic fibrosis, with 55% surviving one year post-operation. Lung function significantly improved after this critical intervention.
Area of Science:
- Pediatric Surgery
- Pulmonology
- Immunology
Background:
- Cystic fibrosis (CF) significantly impacts lung and heart function in children.
- Combined heart-lung transplantation is a potential treatment for end-stage CF.
- Limited data exists on outcomes for pediatric CF patients undergoing this procedure.
Purpose of the Study:
- To evaluate the efficacy and outcomes of combined heart-lung transplantation in pediatric cystic fibrosis patients.
- To assess survival rates, functional improvements, and postoperative complications.
Main Methods:
- Retrospective analysis of 11 children (ages 5-15) with CF undergoing heart-lung transplant between March 1988 and March 1990.
- Immunosuppression regimen included cyclosporine, azathioprine, corticosteroids, and antithymocyte globulin.
- Outcomes assessed included survival, quality of life, pulmonary function (FEV1), and complications.
Main Results:
- 55% of patients (6/11) survived 1.5-23 months with improved quality of life.
- One-year actuarial survival was 55%.
- Mean forced expiratory volume at one second (FEV1) improved from 25% to 73.5% of predicted normal at six months post-transplant.
- Complications included acute rejection (10/11), obliterative bronchiolitis (3 deaths), mediastinitis (1 death), and new-onset diabetes mellitus (4/11).
Conclusions:
- Combined heart-lung transplantation can significantly improve pulmonary function and quality of life in select pediatric CF patients.
- High rates of rejection and significant postoperative morbidities remain challenges.
- Despite complications, the procedure offers a survival benefit for eligible patients with end-stage disease.
Abstract:
From March 1988 to March 1990, 11 children with cystic fibrosis (age 5-15 years) underwent combined heart-lung transplantation at our institutes. Maintenance immunosuppression consisted of cyclosporin and azathioprine with corticosteroids and antithymocyte globulin used perioperatively and during rejection episodes. Six patients (55%) survive from 1.5-23 months all of whom have improved life quality. Actuarial survival to 1 year was 55%. At six months after transplant, mean forced expiratory volume at one second was 73.5% of predicted normal, compared with 25% before transplant. There was one perioperative death, three later deaths associated with obliterative bronchiolitis at two, eight, and nine months, and one from mediastinitis at four months. Of the 15 children accepted for transplantation but not receiving grafts, 10 have died (eight within four months of being placed onto the transplant list). Early postoperative problems included acute reversible rejection (n = 10), meconium ileus equivalent (n = 3), and pancreatitis (n = 1). There was a high incidence of later pulmonary rejection with a mean of 5.7 episodes per patient in the first six months. Pulmonary infection occurred relatively infrequently, with Pseudomonas aeruginosa being the most common pathogen. Persistent diabetes mellitus requiring insulin occurred in four and systemic hypertension developed in one.