Table 1.
Demographics and comparison of different volume/mass groups at baseline
| All groups (n=493) |
LVLM (n=181) |
LVHM (n=78) |
HVLM (n=74) |
HVHM (n=160) |
|
| Demographics | |||||
| Age (years) | 59 (16) | 58*† (15) | 50*§¶ (17) | 67†§** (12) | 59¶** (16) |
| Sex, male/female, n (male %) | 161/332 (33) | 56/125 (31) | 25/53 (32) | 23/51 (31) | 57/103 (36) |
| WHO functional class, n (%) | |||||
| I | 4 (0.8) | 1 (0.6) | 3 (3.8) | 0 (0.0) | 0 (0.0) |
| II | 38 (7.7) | 18 (10.1) | 4 (5.1) | 2 (2.7) | 14 (8.8) |
| III | 384 (78.2) | 152‡ (84.9) | 65 (83.3) | 53 (71.6) | 114‡ (71.3) |
| IV | 65 (13.2) | 8†‡ (4.5) | 6§ (7.7) | 19†§ (25.7) | 32‡ (20.0) |
| PAH subtype, n (%) | |||||
| IPAH | 252 (51.1) | 67*‡ (37.0) | 45* (57.7) | 34** (45.9) | 106‡** (66.3) |
| PAH-CTD | 186 (37.7) | 95*‡ (52.5) | 18*§ (23.1) | 36§** (48.6) | 37‡** (23.1) |
| Other subtypes | 55 (11.2) | 19 (10.5) | 15 (19.2) | 4 (5.4) | 17 (10.6) |
| Treatment regimen, n (%) | |||||
| Monotherapy | 140 (28.7) | 62‡ (34.8) | 23 (29.9) | 21 (28.4) | 34‡ (21.5) |
| Combination | 256 (52.6) | 94 (52.8) | 42 (54.5) | 39 (52.7) | 81 (51.3) |
| Iloprost | 91 (18.7) | 22‡ (12.4) | 12 (15.6) | 14 (18.9) | 43‡ (27.2) |
| Incremental shuttle walking test distance (m) | 219.07 (185.31) | 247.48† (202.99) | 291.79§¶ (209.42) | 125.40†§ (135.68) | 192.02¶(144.89) |
| Right heart catheterisation | |||||
| Mean right atrial pressure (mm Hg) | 10 (6) | 7†‡ (4) | 9§¶ (5) | 12†§ (5) | 13‡¶ (6) |
| Mean pulmonary arterial pressure (mm Hg) | 48 (13) | 40*†‡ (11) | 53*§ (14) | 47†§** (11) | 55‡** (9) |
| Pulmonary arterial wedge pressure (mm Hg) | 10 (3) | 10 (3) | 10 (3) | 11 (3) | 10 (4) |
| Cardiac output (L/min) | 5.0 (1.7) | 5.7*†‡ (1.7) | 4.9* (1.3) | 4.8† (1.9) | 4.4‡ (1.6) |
| Cardiac index (L/min/m2) | 2.8 (0.9) | 3.2*†‡ (0.9) | 2.7* (0.7) | 2.7† (1.0) | 2.5‡ (0.9) |
| Pulmonary vascular resistance (dyn/s) | 687 (394) | 462*†‡ (268) | 770* (391) | 701†** (372) | 906‡** (392) |
| Mixed venous oxygen saturation (%) | 63 (9) | 68*†‡ (7) | 64*¶ (8) | 61† (10) | 59‡¶ (9) |
| CMR imaging | |||||
| RA area (cm2) | 26.82 (10.35) | 21.25*†‡ (7.00) | 24.60*§¶ (8.08) | 29.63†§ (8.77) | 32.89‡¶ (11.42) |
| RVEDVI%pred (%) | 126.25 (44.28) | 94.57†‡ (19.86) | 99.23§¶ (19.90) | 152.33†§ (31.12) | 163.21‡¶ (42.32) |
| RVESVI%pred (%) | 247.66 (122.74) | 147.93†‡ (41.84) | 172.56§¶ (41.71) | 326.79†§** (84.22) | 360.49‡¶** (103.55) |
| RVEF%pred (%) | 56.80 (20.83) | 72.11*†‡ (16.49) | 61.36*§¶ (16.65) | 48.24†§** (14.33) | 41.23‡¶** (15.85) |
| RVSVI%pred (%) | 68.90 (29.60) | 69.02 (21.44) | 61.71§ (21.58) | 74.99§ (30.69) | 69.46 (38.59) |
| LVEDVI%pred (%) | 70.41 (21.89) | 77.75*‡ (21.27) | 63.37*§ (18.38) | 77.41§** (24.13) | 62.35‡** (19.14) |
| LVESVI%pred (%) | 72.65 (33.30) | 71.30† (31.69) | 62.87§ (27.47) | 85.01†§ (39.86) | 73.31 (32.92) |
| LVEF%pred (%) | 98.36 (15.48) | 104.40†‡ (12.83) | 100.51¶ (15.02) | 95.31† (16.65) | 91.89‡¶ (15.11) |
| LVSVI%pred (%) | 50.71 (26.57) | 61.59*‡ (27.45) | 41.05*§ (18.33) | 61.21§** (30.78) | 38.31‡** (18.70) |
| RVEDM (g) | 53.25 (32.20) | 28.64*†‡ (11.36) | 73.49*§ (30.14) | 37.74†§** (13.31) | 78.40‡** (30.03) |
| LVEDM (g) | 90.38 (24.76) | 88.41 (24.85) | 91.38 (26.91) | 95.46 (26.64) | 89.75 (22.46) |
| VMI | 0.59 (0.32) | 0.33*‡ (0.10) | 0.81*§¶ (0.26) | 0.40§** (0.09) | 0.88‡¶ (0.27) |
| RVEDVI%pred:VMI | 262.62 (133.78) | 317.62*†‡ (127.63) | 132.28*§¶ (42.42) | 400.71†§** (114.91) | 200.09‡¶** (73.58) |
| RVESVI%pred:VMI | 487.44 (256.02) | 490.73*† (203.49) | 228.76*§¶ (77.74) | 853.27†§** (242.33) | 440.64¶** (169.49) |
| RV-pulmonary arterial coupling metrics | |||||
| Ea (mm Hg/mL/m2) | 2.07 (1.42) | 1.22*‡ (0.82) | 2.77*§ (1.48) | 1.63§** (1.12) | 2.92‡** (1.41) |
| Ees (mm Hg/mL/m2) | 0.95 (0.43) | 1.20†‡ (0.45) | 1.20§¶ (0.39) | 0.70†§ (0.23) | 0.69‡¶ (0.21) |
| Ees:Ea ratio | 0.80 (0.85) | 1.40*†‡ (1.05) | 0.58*¶ (0.45) | 0.60†** (0.40) | 0.28‡¶** (0.16) |
| CMR Ees:Ea ratio | 0.58 (0.46) | 0.93*†‡ (0.49) | 0.51*¶ (0.34) | 0.44†** (0.27) | 0.23‡¶** (0.13) |
Values are presented as mean (SD) unless otherwise stated.
Treatment data are not available for 6 patients.
*P<0.05 after Bonferroni correction when LVLM was compared with LVHM.
†P<0.05 after Bonferroni correction when LVLM was compared with HVLM.
‡P<0.05 after Bonferroni correction when LVLM was compared with HVHM.
§P<0.05 after Bonferroni correction when LVHM was compared with HVLM.
¶P<0.05 after Bonferroni correction when LVHM was compared with HVHM.
**P<0.05 after Bonferroni correction when HVLM was compared with HVHM.
CMR, cardiac magnetic resonance; Ea, arterial load; Ees, right ventricular elastance; HVHM, high-volume-high-mass; HVLM, high-volume-low-mass; IPAH, idiopathic pulmonary arterial hypertension; LVEDM, left ventricular end-diastolic mass; LVEDVI%pred, left ventricular end-diastolic volume index percentage predicted; LVEF%pred, left ventricular ejection fraction percentage predicted; LVESVI%pred, left ventricular end-systolic volume index percentage predicted; LVHM, low-volume-high-mass; LVLM, low-volume-low-mass; LVSVI%pred, left ventricular stroke volume index predicted; PAH-CTD, connective tissue disease-associated pulmonary arterial hypertension; RA, right atrial; RA, right atrial area; RVEDM, right ventricular end-diastolic mass; RVEDVI%pred, right ventricular end-diastolic volume index percentage predicted; RVEDVI%pred:VMI, ratio of right ventricular end-diastolic volume index percentage predicted to ventricular mass index; RVEF%pred, right ventricular ejection fraction percentage predicted; RVESVI%pred, right ventricular end-systolic volume index percentage predicted; RVESVI%pred:VMI, ratio of right ventricular end-systolic volume index percentage predicted to ventricular mass index; RVSVI%pred, right ventricular stroke volume index percentage predicted; VMI, ventricular mass index.