Although RAP is classically associated with mortality in IPAH (33), two studies, including our smaller previous cohort, showed no association between RAP and mortality in patients with SSc-PAH (7, 47). were white (86.8%) and women (84.2%). The median time of duration of SSc at diagnosis was 10.8 years (range, 0C37 yr), whereas symptoms of Raynaud phenomenon preceded the diagnosis of PAH for a median time of 15 years (range, 0.2C49 yr). The diagnoses of SSc and PAH were established in the same year in 17 patients. TABLE 1. BASELINE CHARACTERISTICS = 0.07). Antitopoisomerase antibodies were present in four patients, all women, all with limited disease. Antinucleolar antibodies were present in 15 (23.8%) patients and were the predominant antibodies in African Americans compared with whites (50% vs. 19%; = 0.07). There were also three patients with antiRNP antibodies, one patient with antiRNA polymerase III, one patient with both anticentromere and antinucleolar antibodies, and six patients with positive undefined antinuclear antibodies. Echocardiographic and hemodynamic data are shown in Table 2. Acetoacetic acid sodium salt Results from baseline echocardiography were available in 65 patients (85%). Forty-four patients (71%) had evidence of RV dilation, and 23 (35%) had evidence of pericardial effusion. Fifteen out of 50 patients (30%) had evidence of nonsystolic dysfunction of the left ventricle. Estimated left ventricular systolic function was normal (mean left ventricular ejection fraction, 60 6%). Traditional hemodynamic measurements indicated moderate-to-severe PAH (mean RAP, 8 4 mm Hg; mPAP, 42 11 mm Hg; cardiac index, 2.4 0.7 L/min/m2; and PVR, 8.6 5.6 Wood units). Mean stroke volume index (31 10 ml/m2) and SV/PP (1.47 0.84 ml/mm Hg) were similarly depressed. TABLE 2. ECHOCARDIOGRAPHIC CHARACTERISTICS AND HEMODYNAMICS = 0.02). TABLE 3. WORLD HEALTH ORGANIZATION FUNCTIONAL CLASS, HEMODYNAMIC VARIABLES, AND FIRST TREATMENT BY YEAR OF DIAGNOSIS = = = = Value= 0.03) and tended to have diffuse SSc subtype (19.3% vs. 8.1%; = 0.28) and systemic hypertension (45.2 vs. 24.3%; = 0.08). Only 2 of the 31 patients with renal dysfunction (6.5%) had a documented previous history of renal crisis. The eGFR was significantly but weakly associated with several baseline hemodynamic parameters, with the following Spearman correlations: PVR, ?0.27; = 0.02; cardiac index, 0.28; = 0.02; Acetoacetic acid sodium salt SVI, 0.39; 0.01; SV/PP, 0.33; 0.01; Acetoacetic acid sodium salt and mixed venous blood oxygen saturation (SvO2), 0.40; 0.01. The correlations with RAP and mPAP were not significant. The proportion of renal dysfunction was higher in patients with WHO FC III to IV (58 vs. 31.2%; = 0.02). The proportion of patients taking diuretics at the time of the analysis was 62.1 and 44.4% for patients with and without renal dysfunction, respectively (= 0.16). Treatment Thirty-eight (64.4%) patients were already receiving or were started on calcium channel blockers at low doses (i.e., 60 mg of nifedipine daily) during follow-up due to different conditions: systemic hypertension, cardiac arrhythmia, or Raynaud symptoms. At least 60 patients (88.2%) received diuretics during follow-up. Sixty-nine (90.8%) patients received PAH-specific therapy after RHC. Initial treatment consisted of intravenous prostacyclin in 8 patients (11.6%), endothelin receptor antagonists in 26 Acetoacetic acid sodium salt (37.7%), phosphodiesterase 5 inhibitors (PDE5-I) in 34 (49.3%), and calcium channel blockers at high dose in 1 (1.4%), all as monotherapy. As shown in Table Adamts1 3, initial therapy varied across the years ( 0.01). At the end of follow-up, 5 patients were on prostanoids alone (7.2%), 10 were on endothelin receptor antagonists alone (14.5%), 19 on PDE5-I alone (27.5%), and 35 patients were receiving combined therapy (50.7%). Survival and Predictors of.