Rationale: Functional studies may be useful to predict survival in idiopathic pulmonary fibrosis (IPF). Various cutoffs of 6-min-walk distance (6MWD) have been suggested to identify patients at a high risk of death. Objectives: To examine the association between 6MWD and survival in patients with IPF listed for lung transplantation, and to identify sensitive and specific cutoffs for predicting death at 6 mo. Patients with a walk distance less than 207 m had a more than fourfold greater mortality rate than those with a walk distance of 207 m or more, despite adjustment for demographics, anthropomorphics, FVC % predicted, pulmonary hypertension, and medical comorbidities (adjusted rate ratio, 4.7; 95% confidence interval, 2.5-8.9; p Ͻ 0.0001). 6MWD was a significantly better predictor of 6-mo mortality than was FVC % predicted (c-statistic ϭ 0.73 vs. 0.59, respectively; p ϭ 0.02). Conclusions: Lower 6MWD was strongly and independently associated with an increased mortality rate for wait-listed patients classified as having IPF. 6MWD was a better predictor of death at 6 mo than was FVC % predicted. Keywords: cohort; exercise test; lung diseases, interstitial; lung transplantationIdiopathic pulmonary fibrosis (IPF) is a devastating disease of unknown etiology characterized histopathologically by usual interstitial pneumonia. Clinically, patients with IPF suffer from progressive respiratory failure, with or without acute exacerbations, and have a median survival of less than 3 yr after diagnosis (1-5). Despite several recent randomized trials, no medical therapy has yet been shown to improve survival in patients with IPF (6-8). Although an uncommon disease with an annual incidence of 7-10 per 100,000 persons, IPF accounts for approximately Lung transplantation at the appropriate point in the disease course may prolong survival in IPF (11); however, the optimal time to proceed with transplantation remains unclear. Previously identified risk factors for death in patients with newly diagnosed IPF include greater age, male gender, lower FVC % predicted, and lower diffusing capacity of carbon monoxide (Dl CO ) % predicted (1, 2, 12-15). Lower peak oxygen consumption during cardiopulmonary exercise testing and oxygen desaturation during 6-min walk testing have also been associated with a higher risk of death in IPF (2,16,17). However, patients with IPF referred and listed for lung transplantation are a distinct cohort. Therefore, extrapolation of survival analyses from patients identified in another context may not be appropriate for decisions regarding the timing of lung transplantation.We recently reported that patients with IPF evaluated for lung transplantation with a 6-min-walk distance (6MWD) less than 350 m had a shorter survival time than those with a 6MWD greater than 350 m (18). Studies of other types of walk tests have shown similar results (19). On the other hand, a recent cohort study of newly diagnosed patients with biopsy-proven idiopathic interstitial pneumonia did not detect an association between 6MWD and...

Both obesity and underweight are independent risk factors for death after lung transplantation, contributing to up to 12% of deaths in the first year after transplantation. Primary care providers and pulmonologists should promote a healthy weight for patients with lung disease long before transplantation is considered.

Outcomes after lung retransplantation have improved; however, retransplantation continues to pose an increased risk of death compared with the initial transplant procedure. Retransplantation early after the initial transplant poses a particularly high mortality risk.

Cardiopulmonary exercise test parameters and the 6MWTD were associated with the risk of death. Measures during exercise may be useful for determination of prognosis and for prioritizing patients with diffuse parenchymal lung disease for lung transplantation.

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