Data on candidate selection and post-transplant follow-up in LAM patients is limited. An NIH-funded a Registry collected data on LAM patients including outcomes of patients undergoing or anticipating lung transplantation. Baseline pulmonary function (PFT) and quality of life data from that population is summarized.
Patients enrolled between 8/98 and 10/01. Six centers registered 243 patients. Initially, patients completed questionnaires detailing medical history, symptoms, laboratory values, treatment, and quality of life. PFTs were done. Follow-ups were at six months and/or yearly.
Of the 243, 13 (5.3%) were transplanted before enrollment (Group A); 21 (8.6%) were transplanted during the Registry period (Group B); 48 (19.8%) were waitlisted or evaluated for transplant during the Registry period (Group C); 161 (66.3%) were not listed or considered for transplant (Group D). Baseline PFTs of Group D and Group A patients were not significantly different; however, baseline PFTs of Group B patients were significantly worse than PFTs of Group A or Group D patients (p=0.015 and <0.001, resp.). Group A patients scored better than Group B patients in the SF-36 physical domain (p=0.003) and in the overall St. George Questionnaire (p=0.006) and it’s activity domain (p<0.001). Group D and Group A scores were similar.
More than one-third of LAM Registry enrollees either had undergone transplant or were considered for transplant.
Pulmonary transplantation appears to be associated with both better pulmonary function and quality of life compared to patients with physiologically advanced disease prior to transplant (Group B). Funded by NIH (NHLBI) Grant No 1 U01 HL58440.
NN*% Pred. FEV1Mean (S.D.)Group A13270.5 (27.6)Group B212046.0 (20.0)Group C484756.7 (19.0)Group D16115177.7 (22.2)
N* = number of patients with FEV1 values
Janet Maurer, None.