Background Lung transplantation is an option for a proportion of patients with end-stage lung disease who deteriorate despite optimal medical therapy. In patients with alpha-1-antitrypsin deficiency (A1ATD), survival benefit has been demonstrated following transplantation. Patient selection is often subjective and the effects of transplantation on Health Related Quality of Life (HRQOL) in A1ATD are unknown. The aims of the current study were to determine objective measures influencing patient selection, survival benefit and health status following lung transplantation in A1ATD patients.
Methods Between 1996 and 2011, patients with A1ATD (PiZ) who had undergone lung transplantation were identified from the UK A1ATD registry. Lung function parameters, including rates of decline pre-transplant, were compared to matched subjects who did not undergo transplantation (matched for sex, age, smoking history and FEV1). HRQOL, assessed using the St George’s Respiratory Questionnaire (SGRQ), was measured pre and post-transplant together with mortality data.
Results Patients who underwent transplantation (n=32) had significantly worse HRQOL than patients who did not in all domains of the SGRQ (total score 64.2; SE±2.5 vs. 55.3±2.04, p=0.008). Markers of gas transfer (TLCO and KCO) were both significantly lower in the transplantation group (33.5%±3.0 and 41.0%±3.9 predicted respectively, compared to 50.6%±.69 and 57.58%±3.1 predicted in the non-transplant group p<0.001 & p=0.001). There were no differences in the rate of lung function decline compared to non-transplanted patients.
Sixteen transplant patients died in the follow up period (6 perioperatively). Figure 1 summarises the Kaplan-Meier survival curve following transplantation. There was no relationship to pre-transplant physiology. Once a survival time of 5 years had been reached, mortality was due to causes unrelated to A1ATD or the transplant.
There was a significant improvement in spirometry (23.94±1.0 vs. 92.73±9.2, p=0.002, N=16) and all domains of the SGRQ post lung transplantation, including the total score (64.2±2.5 vs. 15.6 ±4.9, p=0.002).
Conclusion Patients who underwent lung transplantation had worse gas transfer parameters pre transplant compared to the otherwise matched A1ATD patients who did not. Lung transplantation in patients with end-stage lung disease secondary to A1ATD significantly improved HRQOL.
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