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Transpulmonary gradient (TPG) was defined as mPAP-PAWP and diastolic pressure gradient (DPG) as dPAP-PAWP. Vasoreactivity testing using inhaled nitric oxide was performed on the first RHC. PAWP was measured at end-expiration. Hemodynamic curves obtained from RHC were anonymously reviewed by four Duvelisib mouse specialists (E.B., R.S., V.R. and J.W.H.). Discrepancies in reviewing were resolved by consensus. Patients were followed on a regular basis with a clinical examination every 3?months plus an annual daytime hospitalization with 6MWT, ECG, pulmonary function testing, RHC, TTE and biologic samples. RHC was performed after every dose change of PAH specific treatments. Any acute pathological manifestation would lead to hospitalization, necessary complementary examinations and adaptation of treatment. Long term treatment was started and adapted according to the international guidelines [4]?and?[5]. Patients who had a PAWP >?15?mm?Hg during follow-up and had multiple (��?3) cardiovascular risk factors underwent a coronary angiography. Survival follow-up was performed DNA/RNA Synthesis inhibitor on June 1st, 2012 by telephone interview with the general practitioner or the patient. All-cause mortality was used for analyses. At this time, we divided patients into two different groups: Group A was composed of patients with a strict pre-capillary PAH (i.e. with a measurement of PAWP ��?15?mm?Hg on every RHC performed during follow-up) and Group B included patients who had at least one RHC with a PAWP >?15?mm?Hg during this period of time. As we analyzed changes in group B at first elevation of PAWP, we used the following definitions: high cardiac output (CI?��?3.9?L/min/m2 or CO?��?8?L/min), ventricular interdependence (association of respiratory mitral flow and aortic ejection flow variation, and dilated right ventricle with paradoxal septum found at TTE), coronary artery disease (CAD, defined as one or more significant stenosis ��?70% of a coronary vessel). Statistical analysis was performed using PRDX4 SAS 9.3 Software (SAS Institute, Cary, NC). A p value of