Table 4.
Demographics and Comparison of Incident Treatment-Naive Patients with IPAH and PAH-CTD
| Demographics | IPAH (n = 179) | PAH-CTD (n = 147) | P Value | N |
|---|---|---|---|---|
| Age, yr | 60 (16) | 63 (13) | 0.093 | 326 |
| Sex M/F, n | 73/106 | 31/116 | <0.0001 | 326 |
| WHO functional class, n | ||||
| I | 0 | 1 | ||
| II | 14 | 9 | ||
| III | 128 | 121 | ||
| IV | 137 | 16 | ||
| PAH therapy, n | ||||
| Monotherapy oral | 61 | 49 | 0.888 | 326 |
| Combination oral | 82 | 82 | 0.074 | 326 |
| Prostanoid | 36 | 16 | 0.024 | 326 |
| RHC |
||||
| mPAP, mm Hg | 52 (11) | 43 (12) | <0.0001 | 305 |
| mRAP, mm Hg | 11 (5) | 10 (6) | 0.061 | 303 |
| PAWP, mm Hg | 10 (3) | 10 (3) | 0.207 | 302 |
| SO2, % | 61 (9) | 65 (8) | <0.0001 | 298 |
| CI, L/min/m2 | 2.5 (0.8) | 2.9 (0.8) | <0.0001 | 304 |
| PVRI, Wood units · m2 | 18.1 (7.5) | 13.1 (8.2) | <0.0001 | 299 |
| Cardiac MR indices | ||||
| RVEDVI %pred | 134 (43) | 117 (37) | <0.0001 | 326 |
| RVESVI %pred | 286 (126) | 235 (116) | <0.0001 | 326 |
| RVEF %pred | 48 (18) | 57 (22) | <0.0001 | 326 |
| RVSVI %pred | 63 (19) | 64 (23) | 0.758 | 326 |
| LVEDVI %pred | 63 (19) | 67 (18) | 0.095 | 326 |
| LVESVI %pred | 72 (38) | 67 (18) | 0.136 | 326 |
| LVEF %pred | 93 (16) | 101 (15) | <0.0001 | 326 |
| LVSVI %pred | 41 (21) | 51 (25) | <0.0001 | 326 |
| RVEDMI %pred | 215 (119) | 166 (93) | <0.0001 | 326 |
| PA forward flow index, L/min/m2 | 2.6 (1.0) | 3.0 (0.9) | <0.0001 | 326 |
| PA stiffness metrics | ||||
| PA relative area change | 10 (6) | 11 (8) | 0.089 | 309 |
| PA distensibility | 0.18 (0.15) | 0.31 (0.35) | 0.001 | 300 |
| RV PA coupling metrics | ||||
| Ea, mm Hg/ml/m2 | 1.8 (1.9) | 1.3 (1.3) | 0.003 | 300 |
| Ees, mm Hg/ml/m2 | 0.89 (0.34) | 0.98 (0.43) | 0.047 | 305 |
| Ees/Ea ratio | 0.45 (0.34) | 0.95 (0.84) | <0.0001 | 300 |
| MRI Ees/Ea ratio | 0.54 (0.31) | 0.75 (0.47) | <0.0001 | 326 |
Definition of abbreviations: CI = cardiac index; CTD = connective tissue disease; Ea = arterial load; Ees = RV elastance; IPAH = idiopathic pulmonary arterial hypertension; LVEDVI = left ventricular end-diastolic volume index; LVEF = left ventricular ejection fraction; LVESVI = left ventricular end-systolic volume index; LVSVI = left ventricular stroke volume index; mPAP = mean pulmonary artery pressure; mRAP = mean right atrial pressure; MR = magnetic resonance; MRI = MR imaging; PA = pulmonary artery; PAH = pulmonary arterial hypertension; PAWP = pulmonary arterial wedge pressure; PVRI = pulmonary vascular resistance index; RHC = right heart catheterization; RV = right ventricular; RVEDMI = right ventricular end-diastolic mass index; RVEDVI = right ventricular end-diastolic volume index; RVEF = right ventricular ejection fraction; RVESVI = right ventricular end-systolic volume index; RVSVI = right ventricular stroke volume index; SO2 = mixed venous oxygen saturation; WHO = World Health Organization.
Values are presented as mean (SD) unless otherwise stated.