The “Liver First'' Approach for Combined Lung and Liver Transplantation in Patients with Cystic Fibrosis

Background: Combined lung and liver transplantation is an established therapy for selected patients with cystic fibrosis. The initial sequence for transplantation has primarily been lung first, mainly because it is believed that the lung is more sensitive to prolonged ischemia than the liver. However, this sequence has drawbacks, including the frequent occurrence of primary graft dysfunction (PGD) of the lung, essentially due to coagulopathy, blood loss, transfusion and hemodynamic instability during liver transplantation. Consequently, we changed the sequence to “liver first” in 2006. The aim of this study is to present outcomes of this procedure and to compare them to historic outcomes of combined lung and liver transplantations where the lung was transplanted first.

Methods: The records of combined lung and liver transplant patients treated at our institution between April 1999 and November 2016 were reviewed retrospectively.

Results: A total of 27 patients received a combined lung and liver transplantation. Fifteen patients had cystic fibrosis (CF) as underlying disease. Five CF patients underwent a combined lung and liver transplantation before 2006 beginning with the lung transplant (lung first group). The other ten patients received the liver transplant first, followed by a bilateral sequential lung transplant (liver first group). The lung first group showed a trend toward longer stays on the ICU and in hospital (Median: 17 days in ICU, IQR: 3–47 and 55 in-hospital days, IQR: 29–108) than the liver first group (Median: 6 days in ICU, IQR: 4–19 and 33 in-hospital days, IQR: 26–63). One case of in-hospital mortality was reported in each group. The 90-day, 1-year and 5-year survival rates in the lung first group were 80%, 60% and 20%, respectively. In the liver first group, the survival rates were 90%, 79%, and 79%, respectively. During follow up, three cases of late death were detected in the lung first group, but only one case in the liver first group.

Conclusion: We present the largest series of patients following combined lung and liver transplantation for cystic fibrosis who were transplanted using the liver first approach. The liver first sequence results in favorable outcomes in our cohort of combined lung and liver transplants.