Liver and combined lung and liver transplantation for cystic fibrosis: Analysis of the UNOS database

Abstract

A proportion of patients with CF develop cirrhosis and portal hypertension. LT and combined LLT are rarely performed in patients with CF. To determine the outcome of LT and LLT in patients with CF. Patients with CF who had LT or LLT between 10/1987 and 5/2008 were identified from UNOS database. A total of 182 children (<18 yr) and 48 adults underwent isolated LT for CF. Seven more children and eight adults with CF underwent combined LLT. One- and five-yr patient and graft survival were not significantly different in patients who underwent LT in comparison with patients who underwent LLT (patient survival: LT; 83.9%, 75.7%, LLT; 80%, 80%; graft survival: LT; 76.1%, 67.0%, LLT; 80.0%, 80.0%, respectively). The two major causes of death after LT were pulmonary disease (15 patients, 22.7%) and hemorrhage (12 patients, 18.2%). Bilirubin was identified as a risk factor for death, and previous liver transplant and prolonged cold ischemic time were identified as risk factors for graft loss in LT patients. LT is a viable option for children and young adults with CF and end-stage liver disease. Outcome of LLT patients with CF was comparable to the outcome of patients with CF who underwent isolated LT.

Cystic Fibrosis Liver Disease

“…The difference in mortality was significant in both paediatric and adult populations. Further analysis from UNOS shows good survival at 1 year and 5 years post transplant of 84% and 76% respectively (Arnon et al, 2011). This represents the largest review of outcomes following transplants for CFLD and supports the findings of previous smaller studies (Friedell et al, 2003;Melzi et al, 2006;Nash et al, 2008).…”

“…However, overall survival post liver transplant for CFLD is less than that seen in non-CF patients who receive liver transplants for other indications. The most common cause of long term mortality is progression of pulmonary disease (Friedell et al, 2003), though haemorrhage also represents an important cause of death (Arnon et al, 2011). The criteria and timing for liver transplantation remain uncertain.…”

“…With the shortage of donated organs, this decision needs to be taken carefully. However, survival is comparable for combined liver and lung transplantation where this is indicated (Arnon et al, 2011).…”

Combined lung and liver transplantation: Analysis of a single‐center experience

“…Combined lung and liver transplantation (CLLT) is a viable treatment option for patients with end‐stage lung and liver disease who are not expected to survive with either transplant alone. Diseases that affect both the lungs and the liver, such as cystic fibrosis (CF) and alpha‐1‐antitrypsin deficiency (AATD), have been previously described for CLLT . Putative benefits of multiorgan transplantation with the inclusion of a liver graft include protection against rejection and survivability equivalent to that with single‐organ transplantation .…”

“…The majority of single‐center CLLT cases have been international, with data from the United States arising from multicenter institutions reflected in the United Network for Organ Sharing (UNOS) database. Currently, 58 cases have been published, and the 1‐year mortality rate has ranged from 55.6% to 80% . Patient mortality has been attributed primarily to sepsis, with an earlier study reporting rejection .…”