Table 2 Detailed characteristics of patients with mPAP between 21 and 24 mmHg and PVR ≥ 2 mmHg
Pt # | Patient and SSc characteristics | Clinical characteristics | Lab tests | Chest CT-scan | Pulmonary function tests | ||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Sex | Age (yo) | Time since SSc diag/RP/FEFRPS (y) | SSc type | Antibody profile | mRSS | Tel | RHF | NYHA class | 6MWD (m) (%pred) | Nt-pro-BNP (ng/L) | BNP (ng/L) | Urate (mg/L) | ILD | CTE | PVOD | FVC (% pred) | FEV1 (% pred) | FEV/FVC (%) | TLC (% pred) | DLCO (% pred) | KCO (% pred) | CVF/ DLCO | |
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 | F F F F F F M F F F F F F F M F | 81 71 72 44 82 55 60 65 80 74 52 77 45 65 78 49 | 0/10/0 17/17/17 15/-/- 1/-/1 1/3/2 7/25/17 1/1/1 2/4/4 1/16/1 22/23/23 11/17/16 22/30/22 30/30/27 6/20/20 7/-/- 2/-/- | — lc lc lc dc lc dc lc lc lc lc lc lc lc lc lc | ACA AFA — ACA No spec ACA ATA ACA ACA ACA ACA ATA ACA ACA ACA ACA | — 2 — 2 23 10 26 — 2 — 6 — 4 2 — — | — Yes — Yes No Yes No — Yes — Yes — Yes No Yes Yes | Yes No No No No No Yes Yes No Yes No No No No No No | — II III III II III II III III — II III II II — II | — 273 (49) 210 (38) 372 (63) 330 (73) 426 (66) 307 (62) 348 (59) 325 (69) — 435 (70) 293 (77) 468 (64) 387 (70) — 390 (56) | — 73 — 67 — 135 — — 689 — — 208 — 67 — — | 286 58 130 < 20 114 50 36 — 88 — 57 — 50 59 44 68 | 79 89 87 46 47 44 46 — 74 — 59 — — 54 59 36 | No No No No yes (lim) No yes (ext) yes (ext) yes (lim) — No Yes No No No No | No No — No — No — — No No No — No — No No | — Yes — Yes — No No — No — Yes — Yes — No No | — 98 91 81 113 93 93 80 96 94 115 87 — 120 — — | 40 100 92 65 116 65 94 84 86 82 91 91 — 105 — — | 57 83 109 86 110 76 106 114 100 96 85 112 — 94 — — | — 92 79 86 81 88 78 62 88 — — — — — — — | — 37 22 28 47 44 28 30 50 66 53 71 — 50 — — | — 43 25 27 63 49 31 40 45 59 44 70 — 43 — — | — 2.6 4.1 2.9 2.4 2.1 3.3 2.7 1.9 1.4 2.2 1.2 — 2.4 — — |
2Pt # | ECG | Right-heart catheterization | Echocardiography* | |||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
RAxD | mPAP (mmHg) | PVR (WU) | CO (L/min) | CI (L/min/ m2) | PAWP (mmHg) | RAP (mmHg) | SvO2 (%) | HVPG | Exercise mPAP (mmHg) | Exercise PAWP (mmHg) | Fluid ch mPAP (mmHg) | Fluid ch PAWP (mmHg) | TRV (m/s) | RA area (cm2) | RV/LV ratio | PAAT (ms) | IVC diam (mm) | TAPSE (mm) | RV S’ wave (cm/s) | LVEF (%) | LA vol. index (mL/m2) | E/A ratio | E/e’ ratio | LVFP | Pericard. effusion | |
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 | No No — No No No No No No — No No No No No No | 21 24 21 23 21 22 22 23 23 24 22 24 22 21 21 24 | 3.1 3.3 4.4 3.9 3.5 3.3 3.4 4.8 3.6 2.1 2.0 2.3 2.4 2.6 2.6 2.6 | 3.2 4.3 3.9 4.9 3.7 4.3 4.7 3.8 3.1 4.2 7.2 5.2 6.1 5.0 6.1 8.0 | 2.0 2.6 2.8 2.4 2.6 2.6 2.8 2.3 1.6 2.5 4.2 3.0 3.3 3.3 3.3 4.5 | 11 10 6 4 8 9 6 5 12 14 6 12 7 8 5 3 | 18 5 2 1 7 4 4 4 5 — 6 7 4 5 2 3 | 55 68 55 68 — 71 74 — 63 — 79 73 77 60 76 79 | — — No No — — — — No — No — — — — No | — — — — 22 29 29 38 — 33 — 40 — 37 — — | — — — — — — 6 6 — 19 — 13 — 13 — — | — — — 28 — — — — 28 37 — NA — — — — | — — — 13 — — — — 17 20 — NA — — — — | 2.13 2.70 3.15 3.37 3.2 3.3 — 3.18 2.85 3.05 3.05 2.77 3.1 3.04 3.41 2.0 | 27 11 12 17 14 13 16 12 22 13 16 20 14 16 20 16 | 1.2 0.8 0.7 1.0 0.9 0.7 1.1 — 0.8 0.6 0.8 0.8 0.6 0.7 1.1 0.8 | — 100 77 60 100 150 43 62 81 37 110 104 114 69 78 120 | 24 7.5 17 N 15 19 11 15 10 19 12 11 15 16 10 8 | 20 29 19 21 24 24 22 21 16 21 32 23 30 23 24 29 | — — 10 10 15 11 13 17 9.3 — 10.6 — 12.6 — 15 17.2 | 50 65 45 60 70 65 60 70 60 60 65 70 60 50 65 65 | 23 16 23 18 36 21 18 18 28 21 — 20 — 16 19 16.5 | 0.6 0.7 — 0.7 1.1 1.1 0.7 0.8 3.7 1.4 1.0 0.9 1.3 1.0 0.8 0.8 | — N N 5.0 7.9 15.2 5.5 6.4 4.9 14.5 — 12.2 — 11.6 — — | N N N N N N N N — — — — — — — | No — No No No — — — No — — — — No — — |
Pt # | Screening strategies | Follow-up | ||||||
|---|---|---|---|---|---|---|---|---|
DETECT algorithm | ESC/ERS echocardiographic probability of PH | ASIG algorithm | ESC/ERS risk assessment at baseline (worsening or death at 1 year) | Number of PH drugs (at baseline - during follow-up) | Duration of follow-up (y) | Occurrence of mPAP > 25 mmHg during follow-up (delay from 1st RHC—months) | Death | |
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 | — RHC recommended — RHC recommended RHC recommended RHC recommended RHC recommended — RHC recommended — — — — RHC recommended — — | — Low Intermediate Intermediate Intermediate Intermediate Intermediate Intermediate Intermediate Intermediate Intermediate Low Intermediate Intermediate High Low | — Positive Positive Positive Positive Positive Positive Positive Positive Positive Positive Negative — Positive — — | High risk Intermediate risk High risk Intermediate risk Intermediate risk Intermediate risk High risk High risk High risk Low risk Intermediate risk Intermediate risk Intermediate risk Intermediate risk Intermediate risk Intermediate risk | 0—0 0—0 0—0 1—1 0—0 0—2 0—0 0—1 0—1 0—0 0—2 0—0 0—1 0 -0 0—0 0—3 | 0.3 2.7 0.9 3.4 3.0 4.9 5.3 1.8 3.2 4.3 8.4 3.0 5.4 4.4 2.8 8.0 | — No — Yes (11) Yes (28) Yes (24) Yes (60) Yes (15) Yes (18) — Yes (40) No Yes (60) Yes (50) No Yes (64) | Yes No Yes No Yes No Yes Yes No Yes No Yes No No No No |