Surgery for patients with Alpha 1 Antitrypsin Deficiency: A review

Abstract

Background: Alpha 1 Antitrypsin Deficiency (AATD) is a genetic cause of emphysema/chronic obstructive pulmonary disease (COPD) and liver disease, making AATD patients a high-risk surgical group. Additionally, patients may eventually require lung and/or liver transplantation or lung volume reduction surgery (LVRS). This narrative review discusses perioperative considerations for elective procedures in AATD patients, and reviews patient outcomes in AATD-related transplantation and LVRS.

Data sources: PubMed search terms included: "pre-/peri-/post-operative management"; "COPD"; "AATD"; "lung/liver transplant"; "lung volume reduction."

Conclusions: Lung and liver transplantation in AATD patients are associated with very good long-term survival rates that are comparable to, and sometimes superior to, other transplant indications. Although not currently recommended in AATD, LVRS may have a role in a minority of patients. The value of Alpha 1 Antitrypsin (AAT) augmentation therapy following lung transplantation requires further study. Wherever possible, AAT therapy should be continued in the period around elective surgeries.

Keywords: Alpha 1 Antitrypsin Deficiency; Alpha 1 Antitrypsin augmentation therapy; Liver transplantation; Lung transplantation; Lung volume reduction surgery; Pre-/peri-/post-operative management.