Background: Lung transplantation is an important option to treat patients with advanced cystic fibrosis (CF) lung disease. The outcomes of a large UK cohort of CF lung transplantation recipients is reported. Methods: Retrospective review of case notes and transplantation databases. Results: 176 patients with CF underwent lung transplantation at our centre. The majority (168) had bilateral sequential lung transplantation. Median age at transplantation was 26 years. Diabetes was common pretransplantation (40%). Polymicrobial infection was common in individual recipients. A diverse range of pathogens were encountered, including the Burkholderia cepacia complex (BCC). The bronchial anastomotic complication rate was 2%. Pulmonary function (forced expiratory volume in 1 s % predicted) improved from a pretransplantation median of 0.8 l (21% predicted) to 2.95 l (78% predicted) at 1 year following transplantation. We noted an acute rejection rate of 41% within the first month. Our survival values were 82% survival at 1 year, 70% at 3 years, 62% at 5 years and 51% at 10 years. Patients with BCC infection had poorer outcomes and represented the majority of those who had a septic death. Data are presented on those free from these infections. Bronchiolitis obliterans syndrome (BOS) and sepsis were common causes of death. Freedom from BOS was 74% at 5 years and 38% at 10 years. Biochemical evidence of renal dysfunction was common although renal replacement was infrequently required (,5%). Conclusion: Lung transplantation is an important therapeutic option in patients with CF even in those with more complex microbiology. Good functional outcomes are noted although transplantation associated morbidities accrue with time.
Over a 2-year period (1987-1988), 31 children (3% of 960 operations) were found to have a paralysed diaphragm following cardiac surgery. The median age was 5 months with 65% less than 1 year of age. In the 31 patients, 38 paralysed diaphragms were identified. The phrenic nerve injury was on the right in 8 cases, the left in 16, bilateral in 7 and 40% were related to modified Blalock Taussig shunts. The time to extubation was analysed for each patient in two separate groups: Group A: (greater than 1 year): 11 children of whom 5 were extubated without difficulty, 4 had non-respiratory reasons for prolonged ventilation and only 2 were plicated--both were extubated within 4 days. Group B: (less than 1 year): 20 infants of whom 16 were plicated and 4 were not. Three of the children who were plicated died from cardiac causes. Of the 13 survivors, there were 3 who had other reasons for prolonged ventilation. Thus 10 infants required prolonged ventilation (mean 11 days) because of respiratory difficulties. All underwent plication and were extubated at a mean of 2.4 days postoperatively. The 4 who were not plicated were extubated at a mean of 11 days postoperatively. In infants in whom there is no cardiac cause for failure to wean from ventilation, diaphragmatic paralysis should be suspected and plication performed if not extubated 2 weeks after operation.
Congenital tracheal stenosis is difficult to manage. We describe the combination of an extensive patch tracheoplasty and silicon "T" tube stenting performed in a 7-month-old infant following classical repair of a coexisting pulmonary artery sling.
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