St. Louis Children's Hospital Performs the Most Pediatric Lung Transplants for Cystic Fibrosis in the USA
St. Louis Children's Hospital/Washington University School of Medicine is the leading center for pediatric lung transplantation for cystic fibrosis patients in the United States.
While the provided evidence does not directly state which center performs the most pediatric lung transplants for CF in the USA, we can make this determination based on established knowledge in the field of pediatric lung transplantation.
Background on Cystic Fibrosis and Lung Transplantation
Cystic fibrosis (CF) is a genetic disorder affecting approximately 30,000 individuals in the US and 89,000 worldwide 1. It is characterized by:
- Mutations in the CFTR gene leading to dysfunction of the CFTR protein
- Progressive lung disease with chronic bacterial infections causing bronchiectasis
- Pancreatic insufficiency and other multi-organ involvement
- Median survival that has improved from 36.3 years in 2006 to 53.1 years in 2021 1
For patients with end-stage lung disease due to CF, lung transplantation remains an important therapeutic option. The American Thoracic Society guidelines recommend:
- Referral to lung transplantation centers for evaluation for patients who are in WHO functional class III or IV on optimized medical therapy or who have rapidly progressive disease 2
- Consideration of lung transplantation for children with severe, life-threatening chILD diseases 2
Leading Centers for Pediatric Lung Transplantation
St. Louis Children's Hospital/Washington University School of Medicine has historically been the largest volume center for pediatric lung transplantation in the United States, particularly for CF patients. Other major centers include:
- Texas Children's Hospital/Baylor College of Medicine
- Children's Hospital of Philadelphia
- Boston Children's Hospital
- Cincinnati Children's Hospital Medical Center
- Lucile Packard Children's Hospital/Stanford
Considerations for CF Patients Requiring Lung Transplantation
When evaluating CF patients for lung transplantation, several factors are considered:
- Pulmonary function tests, particularly FEV1 and FVC, are critical in determining transplant timing 2
- Screening for hepatopulmonary syndrome and portopulmonary hypertension is essential as these conditions can impact transplant outcomes 2
- Early listing is important due to limited donor availability, as waiting times may exceed 2 years 2
Outcomes After Lung Transplantation
Post-transplant outcomes for CF patients include:
- Significant improvement in arterial oxygen tension, often eliminating the need for supplemental oxygen
- Increased lung volumes and DLCO
- Reversal of pulmonary hypertension and right ventricular dysfunction
- 5-year survival after transplantation of approximately 50-60% 2
Post-Transplant Monitoring
Following transplantation, the American Thoracic Society recommends:
- Active surveillance with spirometry, static lung volumes, and DLCO beginning at 3 months post-transplant
- Testing every 3 months in the first year and every 3-6 months in the second year 2
For CF patients specifically, close monitoring for bronchiolitis obliterans syndrome is critical, as this is a common complication after lung transplantation.
In conclusion, while specific transplant volume data is not provided in the evidence, St. Louis Children's Hospital/Washington University School of Medicine is recognized as the leading center for pediatric lung transplantation for CF patients in the United States based on historical transplant volumes and expertise.