Prevalence of Pulmonary Hypertension in Patients with Idiopathic Pulmonary Fibrosis: Correlation with Physiological Parameters

Abstract

The aim of this study was to prospectively evaluate the prevalence of pulmonary hypertension (PH) in patients with idiopathic pulmonary fibrosis (IPF). One hundred thirty-nine patients (101 male, mean age = 68.6 ± 9 years), with confirmed IPF and who were admitted to eight Pulmonary Departments in Greece between November 2005 and December 2006 were included in the study. Pulmonary artery systolic pressure (PASP) was estimated by echocardiography, and PH was defined as PASP > 36 mmHg. We compared demographics, pulmonary function tests, NYHA functional status, 6-min walk distance (6MWD), B-type natriuretic peptide (BNP), PaO(2), and P(A-a)O(2) at rest data between patients with PH and without PH (PASP ≤ 36 mmHg). Increased estimated right ventricular systolic pressure was present in 55% of patients (mean PASP = 47.1 ± 11.2 mmHg vs. 30.3 ± 3.8 mmHg, respectively). Patients with PH had a lower but not statistically significant DL(CO) (47.1 ± 18.8 vs. 52.5 ± 20.1), lower PaO(2) at rest (64.6 ± 12.2 vs. 71.1 ± 11.3, P = 0.004), and lower mean 6MWD (282 ± 118 vs. 338 ± 91, P = 0.007). Significant differences were also observed in the NYHA functional status between the two groups (P = 0.02). Statistically significant correlations were observed between PASP and PaO(2) at rest (r = -0.331, P = 0.00), P(A-a)O(2) at rest (r = 0.494, P = 0.00)(,) 6MWD (r = -0.264, P = 0.01), SpO(2) at rest (r = -0.293, P = 0.00), SpO(2) at the end of exercise (r = -0.364, P = 0.00), and also BNP values (r = 0.319, P = 0.01). Moreover, PaO(2) (P = 0.02), P(A-a)O(2) (P = 0.005), and SpO(2) at the end of exercise (P = 0.023) were independent predictors of the presence of estimated PH. Using Doppler echocardiography as a screening tool for the estimation of PH, we found that PH is common in patients with IPF. Gas exchange parameters at rest and exercise desaturation might indicate underlying PH in IPF.

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

“…In general, the prevalence of PH was similar in studies from Europe [18,25,[40][41][42][43][44][45][47][48][49], North America [5,6,26,28,46,[50][51][52][53][54][55][56][57][58][59][60][61][62][63][64][65][66][67], Asia and Africa [37,[68][69][70][71][72][73][74][75], although the majority of evidence was reported from North America (n=23, 53%). Across studies, the prevalence of PH among IPF patients ranged from 3% to 86%; most estimates tended to be between 30% and 50% (figure 3) [25, 43, 44, 47, 50, 52-66, 68, 71-73].…”

Imbalance of circulating endothelial cells and progenitors in idiopathic pulmonary fibrosis

“…On the other hand, these vascular derangements may pave the way for the development of pulmonary hypertension (PH) as a result of irreversible vascular remodeling and increase in vascular resistances [4]. PH is common in IPF even in subjects with no significant functional impairment [9]. It is associated with an increased risk of death [10] and with primary graft dysfunction in transplanted patients [11].…”

Pulmonary Hypertension in Patients with Idiopathic Pulmonary Fibrosis – The Predictive Value of Exercise Capacity and Gas Exchange Efficiency

“…Impaired pulmonary vascular blood flow due to PH results in reduced exercise capacity and disproportional dyspnoea [6], [8], [9], [10]. Peak oxygen uptake (peakVO 2 ) assessed by cardiopulmonary exercise testing (CPET) as well as 6 minute walk distance are significantly impacted by PH [6], [8], [11].…”