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 (%)
 I4 (0.8)1 (0.6)3 (3.8)0 (0.0)0 (0.0)
 II38 (7.7)18 (10.1)4 (5.1)2 (2.7)14 (8.8)
 III384 (78.2)152‡ (84.9)65 (83.3)53 (71.6)114‡ (71.3)
 IV65 (13.2)8†‡ (4.5)6§ (7.7)19†§ (25.7)32‡ (20.0)
PAH subtype, n (%)
 IPAH252 (51.1)67*‡ (37.0)45* (57.7)34** (45.9)106‡** (66.3)
 PAH-CTD186 (37.7)95*‡ (52.5)18*§ (23.1)36§** (48.6)37‡** (23.1)
 Other subtypes55 (11.2)19 (10.5)15 (19.2)4 (5.4)17 (10.6)
Treatment regimen, n (%)
 Monotherapy140 (28.7)62‡ (34.8)23 (29.9)21 (28.4)34‡ (21.5)
 Combination256 (52.6)94 (52.8)42 (54.5)39 (52.7)81 (51.3)
 Iloprost91 (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)
 VMI0.59 (0.32)0.33*‡ (0.10)0.81*§¶ (0.26)0.40§** (0.09)0.88‡¶ (0.27)
 RVEDVI%pred:VMI262.62 (133.78)317.62*†‡ (127.63)132.28*§¶ (42.42)400.71†§** (114.91)200.09‡¶** (73.58)
 RVESVI%pred:VMI487.44 (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 ratio0.80 (0.85)1.40*†‡ (1.05)0.58*¶ (0.45)0.60†** (0.40)0.28‡¶** (0.16)
 CMR Ees:Ea ratio0.58 (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.