Study objectives: To characterize the course of
patients with advanced sarcoidosis who have been listed for lung
transplantation and to identify prognostic factors for death while they
are on the waiting list.
Design: Retrospective cohort
Setting: Tertiary-care university
Patients: Forty-three patients with
sarcoidosis who have been listed for lung transplantation at the
University of Pennsylvania Medical Center.
multivariable explanatory analysis using a Cox proportional hazards
model was performed to determine risk factors that are independently
associated with mortality while patients await transplantation.
Results: Twenty-three of the 43 patients (53%) died while
awaiting transplantation. The survival rate of listed patients (as
determined by the Kaplan-Meier method) was 66% at 1 year, 40% at 2
years, and 31% at 3 years. In a univariate analysis, the following
factors were significantly associated with death on the waiting list:
Pao2 ≤ 60 mm Hg (relative risk [RR], 3.4;
95% confidence interval [CI], 1.2 to 9.3); mean pulmonary artery
pressure ≥ 35 mm Hg (RR, 3.2; 95% CI, 1.1 to 9.5); cardiac index≤
2 L/min/m2 (RR, 2.8; 95% CI, 1.2 to 6.6), and right
atrial pressure (RAP) ≥ 15 mm Hg (RR, 7.6; 95% CI, 3.0 to 19.3).
Multivariable analysis revealed that RAP ≥ 15 mm Hg was the only
independent prognostic variable (RR, 5.2; 95% CI, 1.6 to 16.7;
p = 0.006). Twelve patients underwent lung transplantation. Survival
after transplantation determined by the Kaplan-Meier method was 62% at
both 1 and 2 years, and 50% at 3 years.
Patients with advanced sarcoidosis awaiting lung transplantation have a
high mortality rate with a median survival of < 2 years. Mortality is
most closely linked to elevated RAP. While earlier referral may
diminish the mortality rate of patients on the waiting list for
transplantation, further improvements in posttransplantation outcomes
will be necessary to ensure that this procedure truly bestows a