Lung volume reduction surgery for patients with alpha-1 antitrypsin deficiency emphysema.

Currently, A1AD is recognized in approximately 2% of patients who have emphysema, although this may be an underestimation of the prevalence of this disease. Given the relatively young age at which patients who have A1AD present with emphysema, therapies aimed at slowing the progression of this disease are imperative. In addition to abstaining from smoking, the use of augmentation therapy may benefit some patients who have moderate airflow obstruction. For patients who have severe airflow obstruction, the most effective therapy is surgical. Despite a possible increased risk for infectious complications, transplantation remains a viable option for these patients who have long-term results mirroring those of patients transplanted for smoking-related COPD. Given limited donor availability, however, LVRS must be considered in these patients possibly as definitive therapy but more likely as a bridge to transplantation. LVRS for patients who have A1AD remains relatively uncommon despite a general perception that it remains a surgical option. In a survey of European thoracic surgical centers, Hamacher and colleagues42 found that two thirds of respondents included A1AD in their list of indications for LVRS. Although the durability of the benefits derived from LVRS in patients who have A1AD seems inferior to that of patients who have COPD, the available data show improved 6-minute walk distances and decreased dyspnea persisting for 1 to 2 years after LVRS in patients who had A1AD. Further experience is necessary to determine whether or not subgroups of patients who have A1AD, such as those who have clear heterogeneous distribution, may derive more long-lasting improvement from LVRS.