Alpha 1-Antitrypsin Deficiency: Diagnosis and Replacement in the Lung Transplantation Phase of Life- Survey among Lung Transplant Specialists

Purpose Alpha-1 antitrypsin (AAT) is a significant anti-protease, anti-inflammatory, and immune-regulating protein, which favors organ tolerance after transplantation. Genetic testing for diagnosis of AAT deficiency (AATD) in lung transplant candidates with COPD, and post-transplant replacement of AAT in patients with AATD varies widely among transplants centers. The objective of this survey was to assess the practices of international lung transplant (LTx) centers, pertaining to the testing for, and replacement of, AAT in patients with AATD. Methods We developed a web-based survey to investigate the testing and replacement of AAT in patients with AATD undergoing LTx. The survey was distributed to international LTx centers via the ISHLT Connect platform. Results Respondents consisted of 49 LTx specialists from 10 countries (74% North America, 24% Europe, and 2% Australia). Centers performing >40 transplants per year (considered high-volume) comprised 52% of the respondents. Among all LTx centers, 57% reported screening all patients with COPD for AATD. Screening rates were equal between North America and Europe (58%). Low-volume LTx centers screened for AATD at a much higher rate, compared to high-volume LTx centers (78% vs. 36%). Only 25% of LTx centers provided AAT replacement prior to, or following LTx in patients with known AATD. North America LTx centers replaced AAT (31%) more frequently than European LTx centers (9%). As with screening rates, more low-volume LTx centers provided AAT (35%), compared to high volume centers (16%). One-third of LTx centers reported that, at their respective centers, diagnosis of AATD was an exclusionary criteria for double LTx. Conclusion This is the first systematic survey of LTx centers’ approaches to AATD, with specific attention on testing and post-LTx replacement of AAT. Standards of practice vary widely among lung transplant centers. Low-volume LTx centers screened for AATD, and replaced AAT after LTx in patients with AATD, more frequently than high-volume centers.