Relationship Between α₁-Antitrypsin Deficiency and Ascending Aortic Distention

Objective: To investigate the relationship between α₁-antitrypsin deficiency (AATD), a disorder resulting in protease activity imbalances, and the risk of ascending aortic aneurysm.

Methods: In this single-center study, from August 1, 2018, to February 25, 2019, demographic data were retrospectively collated for patients with AATD-associated emphysema (AATD group) or non-AATD-associated emphysema (control group) with available high-resolution computed tomography scans. Mean ascending aortic diameter was compared between the groups, and the correlation of diameter with age was analyzed.

Results: Patients with AATD (n=51; mean AAT level, 20.3 mg/dL [to convert to μmol/L, multiply by 0.184]) were approximately 10 years younger than those in the control group (n=93; mean AAT level, 172.0 mg/dL), with a mean age of 55 vs 65 years. Overall and grouped by sex, the mean ascending aortic diameter in patients with AATD was not different from that in the control group (overall, 3.34 vs 3.37 cm; P=.68); however, ascending aortic diameter was significantly associated with age for patients in the AATD group (r=0.43; P=.0016), whereas no correlation was observed between age and aortic diameter in the control group (r=0.16; P=.11).

Conclusion: Results of this study suggest that there is a pathologic association between AATD and aortic distention and that AATD may increase the risk of ascending aortic aneurysm. These data provide a basis for the regular assessment of aortic diameter in patients with AATD as well as for the testing of patients with aortic distention or aneurysm for AATD.