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Purpose: The aim of the study was to assess RV remodeling in idiopathic pulmonary arterial hypertensive (IPAH) patients with 3D echocardiography and Cardiac Magnetic Resonance during a follow up of 24 months and to associate remodelling with clinical deterioration.Methods: 30 IPAH patients (mean age: 46.2 ± 13 years) were followed up with conventional 2D echocardiography (2DE), real time 3D echo (3DE) and cardiac MRI (CMR) every 6 months. Six minute walk test distance in every follow up was also recorded. Clinical deterioration was defined as admission to the hospital with signs of right heart failure, deterioration of functional class or death. Agreement between 3DE and CMR was assessed with Bland-Altman agreement analysis.Results: There was an initial improvement of all 2DE indices after initiation of therapy and during the first 6 months of follow up, except right atrial volume which increased despite therapy. RV volumes increased, after 1 year of follow up – as demonstrated with both 3DE and CMR (RV end-diastolic volume-3DE: mean: 168.7 ± 52.7 ml vs. 192.1 ± 51.5 ml (p<0.01)) and RVmass increased within one year, despite medical therapy (RVmass-3DE: mean: 92.4 ± 25.2 gr vs. 114.9 ± 29.3 gr (p<0.01)). There was significant agreement between 3DE and CMR for volumes and ejection fraction (RV end-diastolic volume-baseline: r=0.85, mean bias = -15 ml, SD of bias= 30 ml – RV ejection fraction: r = 0.8, mean bias = 0.9%, SD of bias = 7.2% ) but not for RV mass (baseline: r= -0.27, p=0.14). From the total number of patients, 8 patients (26%) clinically deteriorated. The group of the patients which deteriorated was compared to the patients who remained stable, and univariate analysis demonstrated that the first group demonstrated a further increase of volumes (RVEDV and RVESV), reduction of RVEF and a regression of RV mass after 19 months from initial diagnosis. More precisely, stable patients had an RVEDV less than 200 mls throughout follow up, while patients who clinically deteriorated further increased RVEDV. RVESV was increased for both groups, however it increased more rapidly in patients who clinically deteriorated. An RVEF less than 23% together with regression of RV mass indicated clinical deterioration.Conclusion: In IPAH patients, RV volumes dilate and ejection fraction decreases over time. 3DE and CMR have significant agreement for volumes and ejection fraction but there are important limitations for the measurement of RV mass. Clinical deterioration may be indicated by a low ejection fraction and a regression in RV mass.