Abstract
Introduction: In patients with Idiopathic Pulmonary Arterial Hypertension (IPAH) a wide range of diffusion capacity for carbon monoxide (DLCO) values can be observed. Low DLCO in IPAH is associated with worse prognosis when compared to normal DLCO1.
Hypothesis: histopathological pattern(s) of pulmonary vasculopathy in IPAH differ between patients with low and normal DLCO.
Methods: We retrospectively analyzed the histopathological (combinations of) patterns of vasculopathy in IPAH patients with low and normal DLCO. Low DLCO was defined as lowest tertile of the bimodal distribution of DLCO-value in a series of 170 IPAH patients1 (<45% pred.). DLCO in the upper 2 tertiles (≥ 45 % pred.) was defined as normal DLCO.
Results: Out of 170 IPAH patients, complete data sets were obtained in 20 patients: low DLCO N=10; normal DLCO N=10. Results are shown in table 1 (study is ongoing).
Conclusion: A PVOD/PCH-like pattern is more common in IPAH with low DLCO, while plexogenic arteriopathy prevails in IPAH with normal DLCO.
1. Trip et al, abstract this meeting.
- © 2012 ERS