The spectrum of systemic sclerosis-associated pulmonary hypertension (SSc-PH): insights from the ASPIRE registry

<bold>Introduction:</bold> There are few published data comparing different forms of SSc-PH <bold>Methods:</bold> Data for 912 SSc patients assessed 2000-20 were retrieved from the ASPIRE registry <bold>Results:</bold> Precapillary PH diagnoses ↑ significantly with ↓ PVR threhold to 2WU: ESC2015 (mPAP≥25,PVR>3) n=503; 6thWSPH (mPAP>20,PVR>3) n=519; ESC2022 (mPAP>20,PVR >2) n=615 PAH/PH-Lung: PH-Lung survival was inferior to PAH (2.7v4yrs, p=0.03). Median ΔISWD following PAH treatment was 0m, however patients who ↑ISWD by ≥30m had superior survival Unclassified-PH: Survival was similar to No-PH. PAWP was higher than in pre-capillary PH while LA size was similar to PH-LHD. 23% developed PAH and 13% PH-LHD at repeat RHC. PVOD: The 5% of PAH patients with 2-3 radiological features of PVOD had higher mPAP, lower DLco and worse survival compared to those with 0-1 features. PH-LHD: Survival was similar to PAH. IpcPH 5-yr survival was 72% and CpcPH 39%. LA volume in PAWP 13-15 was > than in PAWP ≤12 but similar to PH-LHD. <bold>Conclusion:</bold> Characteristics and outcomes differ across the SSc-PH spectrum. <table-wrap><object-id>erj;64/suppl_68/PA2492/TB1</object-id><object-id>T1</object-id><object-id>TB1</object-id><table><colgroup><col></col><col></col><col></col><col></col><col></col><col></col><col></col></colgroup><tbody><tr><td></td><td><bold>PAH</bold></td><td><bold>PH-Left Heart</bold></td><td><bold>PH-Lung</bold></td><td><bold>Unclassified-PH</bold></td><td><bold>No-PH</bold></td><td><bold>p</bold></td></tr><tr><td><bold>N</bold></td><td><bold>386</bold></td><td><bold>61</bold></td><td><bold>230</bold></td><td><bold>39</bold></td><td><bold>99</bold></td><td></td></tr><tr><td><bold>FVC%</bold></td><td>100±22</td><td>78±26</td><td>79±25</td><td>100±23</td><td>97±22</td><td><0.001</td></tr><tr><td><bold>DLco%</bold></td><td>41±12</td><td>43±16</td><td>32±12</td><td>57±15</td><td>55±17</td><td><0.001</td></tr><tr><td><bold>mPAP</bold></td><td>40±13</td><td>39±14</td><td>38±12</td><td>24±2</td><td>17±2</td><td><0.001</td></tr><tr><td><bold>PAWP</bold></td><td>10±3</td><td>18±3</td><td>9±3</td><td>13±2</td><td>8±3</td><td><0.001</td></tr><tr><td><bold>PVR</bold></td><td>8±6</td><td>5±5</td><td>7±5</td><td>1.7±0.3</td><td>2±1</td><td><0.001</td></tr><tr><td><bold>CMR:RVEF</bold></td><td>42±12</td><td>47±15</td><td>40±12</td><td>56±7</td><td>51±10</td><td><0.001</td></tr><tr><td><bold>CMR:LA Volume</bold></td><td>69±26</td><td>86±23</td><td>62±22</td><td>98±29</td><td>66±32</td><td><0.001</td></tr></tbody></table></table-wrap> <fig><object-id>erj;64/suppl_68/PA2492/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>