Our hypothesis is that optimal treatment of the dysfunctional metabolic pathways which underlie PAH will improve pulmonary vascular function and consequences of the disease.
This study will help to provide more up to date prognostic data on the natural history of untreated proximal CTEPH which will be helpful in discussions regarding surgical versus medical treatment and by exploring the similarities and differences between distal CTEPH and IPAH the investigators hope to get insights into disease mechanisms in patients with distal CTEPH.
Start: September 2012
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