Lung transplantation in alpha 1 antitrypsin deficiency

Survival benefit has been demonstrated following lung transplantation in alpha-1-antitrypsin deficiency (A1ATD)The aims of this study were to determine survival benefit and health status following lung transplantation in A1ATD patients compared to a closely matched control group. Patients with PiZ A1ATD who had undergone lung transplantation were identified from the UK registry.Survival was compared to matched subjects who did not undergo transplantation (matched for sex, age, smoking history, FEV 1 gas transfer and health status). It was only possible to match 30 transplant patients for all parameters to patients who did not undergo transplantation.Survival in the 2 patient groups was similar after those who died in the immediate post-op period (90 days) were excluded. There was a significant improvement in spirometry (FEV 1 rising from 23.9 to 92.7 p0.002) and all domains of the SGRQ post transplant (total score 64.2 vs. 15.6 p0.002) in the patients who survived the post-op period. There is clear improvement in HRQoL following lung transplantation in patients with A1ATD.Gas transfer and HRQoL are not usually measured as part of a transplant assessment and studies have considered FEV 1 as a key measure of suitability for transplantation.Matching to a nontransplant cohort by this alone may not provide robust data on survival that many depend on health status and gas transfer.Survival was not significantly different from a carefully matched non-transplant cohort.