Abstract
Introduction: Lung diffusion capacity for carbon monoxide (DLCO) impairment is often in IPAH patients (pts), and it can be profound in some of them. Objectives: To assess the degree of DLCO impairment, its correlates with other parameters, and its prognostic significance in IPAH. Methods: 65 IPAH pts (49 women and 16 men, median age 43 years) were allocated to one of two groups depending on DLCO% pred.value. Pts with DLCO Results: DLCO<55% pred. was found in 18% of pts. In comparison to pts with DLCO≥55% pred. they had: more pronounced disturbances in breathing gas distribution: MMEF% pred. 44 vs 76 p=0.008, VA/TLC 0.8 vs 0.88 p=0.01; higher proportion of WHO FC class III and IV: 75% vs 26% p=0.005; shorter 6MWT distance: 284m vs 395m p=0.01, and lower minimal saturation during exercise: 82% vs. 94% p=0,0006. There were no significant differences in hemodynamic profile. Low DLCO was a negative prognostic indicator for survival and carried a 4-fold increase of death risk in 5-year perspective (HR 3.9, 95%CI:1.8-8.6, p= 0.0006). Conclusions: DLCO correlated well with functional but not hemodynamic parameters in IPAH. Low DLCO was associated with worse prognosis.
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