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Left Lung Orthotopic Transplantation in a Juvenile Porcine Model for ESLP
Published on: February 14, 2022
Lung transplantation for childhood diffuse lung disease
Jennifer A Rama1, Leland L Fan, Albert Faro
1Pediatric Pulmonary Medicine, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas 77030, USA. jarama@texaschildrens.org
Insights
Pediatric lung transplantation outcomes for diffuse lung disease (DLD) are comparable to cystic fibrosis and pulmonary vascular disease. DLD patients experienced higher pre-transplant morbidity but similar post-transplant survival rates.
Area of Science:
- Pediatric Pulmonology
- Thoracic Surgery
- Transplantation Medicine
Background:
- Pediatric diffuse lung diseases (DLD) are rare, heterogeneous disorders.
- These conditions can lead to end-stage lung disease requiring lung transplantation.
- Limited data exists on DLD outcomes due to small patient cohorts.
Purpose of the Study:
- To compare pre- and post-transplant factors in pediatric patients with DLD undergoing lung transplantation.
- To evaluate DLD outcomes against other end-stage lung disorders like cystic fibrosis (CF) and pulmonary vascular disease (PVD).
Main Methods:
- Retrospective chart review of lung transplant recipients from 2002-2007 at two major pediatric centers.
- Comparison of pre-transplant characteristics and post-transplant outcomes among DLD, CF, and PVD groups.
Main Results:
- DLD patients showed higher pre-transplant morbidity, including lower FEV1, pulmonary hypertension, and hypercapnia.
- More DLD and PVD patients required intensive care unit (ICU) admission and mechanical ventilation compared to CF patients.
- While acute rejection rates differed, survival, infection rates, and post-transplant lymphoproliferative disease were similar across groups.
Conclusions:
- Lung transplantation is a successful treatment option for pediatric patients with end-stage diffuse lung disease.
- Outcomes for DLD patients are comparable to those with cystic fibrosis and pulmonary vascular disease post-transplantation.
Background:
Pediatric diffuse lung diseases comprise a heterogeneous group of rare lung disorders which may lead to end stage lung disease and referral for lung transplantation. Previous studies are limited by small numbers of patients with specific forms of diffuse lung disease. Children with all forms of diffuse lung disease who underwent lung transplantation at two pediatric centers were evaluated in terms of several pre- and post-transplant factors and compared to children with other end stage lung disorders.
Methods:
A retrospective chart review was performed on all patients transplanted between October 1, 2002 and June 15, 2007 at Texas Children's Hospital and St. Louis Children's Hospital. Multiple pre-transplant characteristics and post-transplant morbidities and mortality were compared between diffuse lung disease, cystic fibrosis, and pulmonary vascular disease groups.
Results:
There were 31 diffuse lung disease (DLD), 57 cystic fibrosis (CF), and 16 pulmonary vascular disease (PVD) patients included in our analysis. Patients with DLD had significantly higher pre-transplant morbidity including lower percent predicted of forced expiratory volume in first second (P = 0.013) and more patients with pulmonary hypertension (P = 0.001) and hypercapnia (P = 0.031). Compared to CF patients, more DLD and PVD patients required invasive ventilation (P = 0.001) and care in the pediatric intensive care unit (P = 0.001). After transplant, there was a difference among the three groups with regards to number of acute allograft rejections but statistical limitations preclude knowing between which group the difference lies. A difference in time to bronchiolitis obliterans was found between the PVD and CF groups but not when compared to the DLD patients. The three groups had similar time to post-transplant lymphoproliferative disease, rate of infections, and survival.
Conclusion:
Lung transplantation is as successful for patients with end stage diffuse lung disease as compared to other lung transplant candidates.
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