What is the role of volume-reduction surgery in the treatment of alpha1-antitrypsin deficiency (AATD)?

Updated: Sep 11, 2020
  • Author: Dora E Izaguirre Anariba, MD, MPH; Chief Editor: John J Oppenheimer, MD  more...
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Answer

Two surgical approaches may help selected patients with emphysema due to alpha1-antitrypsin deficiency (AATD).

Volume-reduction surgery in AAT deficiency

Volume-reduction surgery has generated nationwide interest and hope for patients with all types of emphysema. [20]

Selected patients with severe emphysema and significant air trapping have experienced symptomatic improvement by removing the most severely affected 20-35% of each lung. Spirometry and exercise tolerance generally improve following postoperative recovery. Dyspnea generally is diminished. The effects on blood gas values are variable.

Some of the enthusiasm for the procedure has waned, even as surgical mortality rates have diminished, because the duration of improvement seems to be brief; an accelerated rate of FEV1 decline appears to occur after the surgery.

The randomized controlled National Emphysema Treatment Trial showed benefit to only those with poor exercise tolerance and predominantly upper lobe disease. Others with diffuse disease, basilar disease, and/or good exercise tolerance did not benefit from lung-volume reduction. In some instances, mortality was increased. This study included patients with emphysema of all etiologies.

A small prospective study of 21 patients with alpha1-antitrypsin deficiency showed improvement in the mean dyspnea score at 3 months after surgery. This finding persisted for as long as 3.5 years. Improvements were also noted in mean FEV1, vital capacity, and the ratio of residual volume to total lung capacity; these results persisted for 1-2 years. Patients with heterogeneous emphysema with little or no inflammatory airway disease appeared to benefit most. Overall, changes in patients with advanced emphysema from alpha1-antitrypsin deficiency were inferior to those changes in patients with smoking-related emphysema, as they were decreased in magnitude and duration.


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