PURPOSE: Pulmonary hypertension (PH) is a major cause of death in systemic sclerosis (SSc). The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) is a prospective longitudinal study of patients with PH and those at high risk of developing PH. Currently, WHO group I classify patients with mean pulmonary artery pressure (mPA) < 20 mmHg is “normal” and 21-24 mmHg is “borderline” PH. The purpose was to compare baseline demographics in normal SSc patients vs. borderline PH from PHAROS.
METHODS: SSc patients at high risk for PH included a DLCO<55% predicted, FVC%/DLCO% >1.6, and/or RVSP > 35.
RESULTS: 67 of 238 high risk SSc patients underwent RHC with mPA < 25. Of these 67, 39 (58%) were ‘normal’ (mPA<20 mmHg) and 28 (42%) had ‘borderline’ PH (mPA 21-24 mmHg). Average age 55.3yrs, 85% women; 66% limited, and time to first non-Raynaud’ s symptom was 8.8yrs. No differences were noted in baseline demographics between groups. Compared to ‘normal’ , ‘borderline’ had lower FVC% (70 vs. 81, p< 0.05), and trended to have lower DLCO%, and higher FVC%/DLCO%. There were no differences in patient reported outcomes between groups. More patients with ‘borderline’ PH had NYHA III compared to ‘normal’ (28% vs. 10%), and a lower 6MW (p>0.05). ‘Borderline’ PH had a significantly higher PVR, and transpulmonary gradient (TPG) compared to ‘normal’ , p<0.05. Half of the ‘borderline’ group had an abnormal PVR compared to 8% of ‘normal’ (p< 0.003). On exercise, ‘borderline’ were more likely to have mPA>30 than ‘normal’ , 11/13 (85%) vs. 11/19 (58%).On follow up 4 ‘borderline’ patients had repeat RHC, 1 had PAH and 3 had pulmonary venous hypertension (PVH).
CONCLUSION: Preliminary analysis demonstrates SSc with ‘borderline’ PH are more likely to have restrictive lung disease, greater decrements in functional class, higher PVR and TPG, and abnormal response to exercise.
CLINICAL IMPLICATIONS: Investigation on the prognostic and therapeutic implications of such findings in ‘borderline’ PH are warranted.
DISCLOSURE: Rajeev Saggar, No Financial Disclosure Information; No Product/Research Disclosure Information