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Published on: March 8, 2019
Relationship Between α1-Antitrypsin Deficiency and Ascending Aortic Distention
Farouk Dako1, Huaqing Zhao2, Alexandra Mulvenna3
1Department of Radiology, University of Pennsylvania, Philadelphia, PA.
Alpha-1 antitrypsin deficiency (AATD) is linked to aortic distention, potentially increasing ascending aortic aneurysm risk. Regular aortic diameter assessment is recommended for AATD patients.
Area of Science:
- Cardiovascular Medicine
- Pulmonology
- Genetics
Background:
- Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder characterized by low levels of alpha-1 antitrypsin (AAT), leading to protease-antiprotease imbalance.
- This imbalance is primarily associated with lung disease (emphysema) but may have systemic implications.
- The potential link between AATD and aortic pathology, specifically ascending aortic aneurysm, remains underexplored.
Purpose of the Study:
- To investigate the association between alpha-1 antitrypsin deficiency (AATD) and the risk of developing ascending aortic aneurysms.
- To compare the mean ascending aortic diameter in patients with AATD-associated emphysema versus those with non-AATD emphysema.
Main Methods:
- Retrospective, single-center study analyzing demographic data and high-resolution computed tomography (HRCT) scans.
- Inclusion criteria: patients diagnosed with AATD-associated emphysema (AATD group) or non-AATD emphysema (control group).
- Comparison of mean ascending aortic diameter between groups and analysis of the correlation between aortic diameter and age.
Main Results:
- Patients with AATD were younger (mean age 55 years) than controls (mean age 65 years).
- No significant difference in mean ascending aortic diameter was observed between the AATD group (3.34 cm) and the control group (3.37 cm).
- Ascending aortic diameter significantly correlated with age in the AATD group (r=0.43, P=.0016) but not in the control group (r=0.16, P=.11).
Conclusions:
- Findings suggest a pathological association between AATD and aortic distention.
- AATD may represent an independent risk factor for ascending aortic aneurysm development.
- Recommend regular aortic diameter screening in AATD patients and AATD testing in patients with aortic distention or aneurysms.
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