Liver and lung transplantation in cystic fibrosis: an adult cystic fibrosis centre's experience

Sivam, Sheila; Al-Hindawi, Y.; Michiel, James Di; Moriarty, Carmel; Spratt, P.; Jansz, P.; Malouf, M.A.; Plit, M.; Pleass, Henry; Havryk, A.; Bowen, David; Haber, Paul; Glanville, Allan R.; Bye, Peter

doi:10.1111/imj.13126

Cited by 4 publications

(1 citation statement)

References 11 publications

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“…In addition, four other patients were undergoing evaluation for lung transplantation. 56 Taken together, while liver transplantation may slow decline in pulmonary function, it appears not to halt the disease process and patients may ultimately require lung transplantation as well.…”

Section: Transplantationmentioning

confidence: 99%

Hepatic Manifestations of Cystic Fibrosis

Sakiani

Kleiner

Heller

et al. 2019

Clinics in Liver Disease

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Section: Transplantationmentioning

confidence: 99%

Hepatic Manifestations of Cystic Fibrosis

Sakiani

Kleiner

Heller

et al. 2019

Clinics in Liver Disease

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Liver-first strategy for a combined lung and liver transplant in patients with cystic fibrosis

Aburahma

Salman

Engel

et al. 2021

European Journal of Cardio-Thoracic Surgery

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OBJECTIVES A combined lung and liver transplant in patients with cystic fibrosis (CF) is an uncommon procedure. The goal of this study was to compare long-term outcomes between patients with CF who underwent either a combined lung-liver or a lung-only transplant. METHODS This is a retrospective single-centre study of patients with CF who underwent a lung transplant between January 2005 and May 2020. Since 2006, our preference for a combined lung-liver transplant was to transplant the liver first and then the lung. Outcomes were compared using the Kaplan–Meier analysis and the log-rank test. Median follow-up was 53 (23–97) months. RESULTS During the study period, among 357 patients with CF who underwent a lung transplant, 14 (4%) required a lung-liver transplant whereas 343 (96%) had a lung-only transplant. Lung cold ischaemic time was longer in the lung-liver transplant group, but no patient in this group showed primary graft dysfunction at 72 h after the transplant. Prevalence of anti-human leucocyte antigen donor-specific antibodies was 7.1% vs 13.7% in the lung-liver versus the lung-only transplant group (P = 0.42). At 5 years, lung graft survival (78% vs 69%) and freedom from chronic lung allograft dysfunction (79% vs 62%) did not differ between the lung-liver versus the lung-only groups (P = 0.45 and P = 0.55, respectively). Freedom from lung biopsy-confirmed rejection was significantly higher in patients undergoing a lung-liver transplant (91% vs 50%; P = 0.027). CONCLUSIONS A lung-liver transplant did not impair lung graft function. The lower prevalence of donor-specific antibodies and the better freedom from lung biopsy-confirmed rejection suggest tolerogenic effects of the liver graft.

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Conquering combined thoracic organ and liver transplantation

Yi¹,

Lunsford²,

Bruce

et al. 2018

Current Opinion in Organ Transplantation

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Combined thoracic and liver transplant continues to be a viable treatment option for patients with end-stage disease that would likely not survive single transplant alone. Salvage modalities, such as extracorporeal membrane oxygenation and ex-vivo lung perfusion, may help in extending candidacy for this combined transplant. Outcomes, to date, are similar to results observed for solitary thoracic organ recipients, justifying CHLT and CLLT as a viable option for these patients. Continued identification of outcomes is needed to justify allocation of dual organs to a single recipient.

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Liver and lung transplantation in cystic fibrosis: an adult cystic fibrosis centre's experience

Cited by 4 publications

References 11 publications

Hepatic Manifestations of Cystic Fibrosis

Hepatic Manifestations of Cystic Fibrosis

Liver-first strategy for a combined lung and liver transplant in patients with cystic fibrosis

Conquering combined thoracic organ and liver transplantation

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