Surgical and Bronchoscopic Lung Volume Reduction Treatment for α-1 Antitrypsin Deficiency

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α-1 antitrypsin deficiency (AATD) is an underrecognized cause of emphysema with associated hyperinflation and compromised quality of life. Lung volume reduction surgery for AATD has been reported in a small number of series. In general, the magnitude and duration of benefit of lung volume reduction surgery for AATD is smaller and briefer in AATD than in α-1 antitrypsin-replete chronic obstructive pulmonary disease. Nonsurgical approaches to lung volume reduction have included various endobronchial approaches, including valves, glue, and steam. Endobronchial valves have been shown to confer benefit in chronic obstructive pulmonary disease, with the largest available controlled trial (VENT) showing enhanced FEV1 and 6-minute walk distance in valve recipients, albeit at the expense of more complications. Three small series examining a total of 29 patients have reported use of endobronchial valves in individuals with AATD, again suggesting benefit. To our knowledge, no other nonsurgical approaches to lung volume reduction have been reported in AAT-deficient patients. Overall, on the basis of a small current experience, endobronchial treatment to effect lung volume reduction in AATD appears promising, with further study needed to clarify its role and regulatory approval.

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