To evaluate the effect of pulmonary hypertension (PH) on survival of patients with idiopathic pulmonary fibrosis (IPF) .
We identified 88 patients who had transthoracic echocardiograms with measurement of systolic pulmonary artery pressure (SPAP) performed within 3 months of initial evaluation for IPF at Mayo Clinic (index visit) from 1994 through 1996.
Eighty-eight patients were stratified into three groups based on their estimated SPAP: SPAP < or = 35 mmHg, 35< SPAP< or = 50 mmHg, and SPAP >50 mmHg: 14, 47 and 27 patients respectively. The estimated SPAP ranged from 27.7 to 116.0 mmHg (mean 48.3±15.5). As SPAP increased, patients were more likely to die with a hazard ratio of 1.34 for each 10-mmHg increase in SPAP (p<0.001). When comparing survival across SPAP groups, the SPAP>50 had a significantly worse survival compared to the others (p=0.009). The estimated 1 and 3-year survival along with the median for these 3 groups were: 0> SPAP < or = 35: 1 year=100%; 3 year=64%; median=4.8 years 35< SPAP < or = 50: 1 year= 79%; 3 year=61%; median=4.1 years SPAP >50: 1 year= 44%; 3 year=32%; median=0.7 years. From preliminary univariate analysis, for the group as a whole, survival was worse with higher SPAP (p<0.001, RR 1.34 per 10 mmHg increase), male gender (p = 0.004, RR = 1.7), lower DLCO (p < 0.001, RR = 0.62 per 10 percentage points), and known coronary artery disease (p=0.017, RR = 1.96) .
Pulmonary hypertension, particularly SPAP> 50 mmHg, is associated with worse survival in patients with IPF. The prevalence and prognostic significance of pulmonary hypertension in patients with IPF as well as impact of therapy for pulmonary hypertension on these patients requires further exploration.
1. The presence of pulmonary hypertension portends a worse prognosis in patients with IPF. 2. Echocardiographic evaluation should be considered in the clinical assessment of patients with IPF. 3. Treatment of pulmonary hypertension may prolong survival in patients with IPF.
H.F. Nadrous, None.