Alpha 1-antitrypsin deficiency surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]

Overview

Lung transplantation may be recommended for some patients with end-stage lung disease. Alpha 1-antitrypsin deficiency accounts for about 5% of all lung transplantation performed in the United States. Five year survival rates following lung transplant is approximately 50%. The rate of FEV1 decline among AATD patients who received double lung transplantation was faster than among single lung recipients. The estimated median survival time was 11 years in transplant recipients versus 5 years in controls. Lung volume reduction surgery (LVRS) can help relieve dyspnea and improve exercise capacity in patients with emphysema. Data regarding the efficacy of LVRS for individuals with AATD is limited and generally less favorable in magnitude and duration of FEV1 improvement.

Lung Transplantation

  • Lung transplantation may be recommended for some patients with end-stage lung disease.[1][2]
  • Alpha 1-antitrypsin deficiency accounts for about 5% of all lung transplantations performed in the United States.
  • Single lung transplant is more common despite the fact that outcome is better in patient who receive double lung transplant due to limitations on availability.
  • Five year survival rates following lung transplant is approximately 50%.
  • Bronchiolitis obliterans being the major cause of death post-transplant.
  • Outcomes of lung transplantation can vary considerably.
  • For example, reports from Washington University Medical Center in St. Louis, the Copenhagen National Lung Transplant Group, and Zurich University Hospital reported no difference in 5-year survival rates between AATD-COPD and usual COPD recipients.
  • In contrast, the Toronto group reported that AATD patients had lower 10-year survival (23% vs 43%) compared with usual COPD patients.
  • A recent study from the 2 national lung transplantation centers in Sweden documented a median survival time for AATD-COPD patients of 12 years compared with only 6 years for usual COPD patients.
  • The rate of FEV1 decline among AATD patients who received double lung transplantation was faster than among single lung recipients.
  • This difference persisted after adjustment for age, smoking status, body mass index, oxygen use, exercise capacity, donor age, cytomegalovirus mismatch, and transplant type.
  • The estimated median survival time was 11 years in transplant recipients versus 5 years in controls.

Lung Volume Reduction Surgery

  • Lung volume reduction surgery (LVRS) can help relieve dyspnea and improve exercise capacity in patients with emphysema. Data regarding the efficacy of LVRS for individuals with AATD is limited and generally less favorable in magnitude and duration of FEV1 improvement.
  • One study demonstrated that bilateral LVRS in AAT-deficient patients with emphysema can be beneficial but functional measurements except 6 min walk test, returned to baseline at 6–12 months. Although, LVRS was not recommended for management of AAT deficiency.

References

  1. Glanville AR, Estenne M (2003). "Indications, patient selection and timing of referral for lung transplantation". Eur. Respir. J. 22 (5): 845–52. PMID 14621095.
  2. Thabut G, Christie JD, Ravaud P, Castier Y, Dauriat G, Jebrak G, Fournier M, Lesèche G, Porcher R, Mal H (2009). "Survival after bilateral versus single-lung transplantation for idiopathic pulmonary fibrosis". Ann. Intern. Med. 151 (11): 767–74. doi:10.7326/0003-4819-151-11-200912010-00004. PMID 19949142.



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