Table 1.
Overview of Baseline Characteristics Stratified by Event‐Free Survival
| Demographics and clinical characteristics | Entire cohort | Event‐free patients | Patients with death or transplantation | P value |
|---|---|---|---|---|
| Number of patients | 63 | 42 | 21 | |
| Male sex, n (%) | 25 (39.7) | 16 (38.1) | 9 (42.9) | 0.93 |
| Age at baseline, y, mean (SD) | 9.05 (5.24) | 8.86 (5.35) | 9.44 (5.13) | 0.68 |
| Diagnosis, n (%) | 0.78 | |||
| Associated PHT | 21 (33.3) | 0 (0) | 6 (28.6) | |
| Idiopathic PAH | 42 (66.7) | 27 (100) | 15 (72.5) | |
| Weight, kg, mean (SD) | 28.61 (15.36) | 28.62 (14.35) | 28.60 (17.58) | 1.00 |
| Height, cm, mean (SD) | 125.20 (31.00) | 124.90 (32.19) | 125.85 (29.13) | 0.91 |
| WHO‐FC, n (%) | 0.01 | |||
| I | 6 (10.0) | 5 (12.5) | 1 (5.0) | |
| II | 26 (43.3) | 22 (55.0) | 4 (20) | |
| III | 27 (45) | 13 (32.5) | 14 (70) | |
| IV | 1 (1.7) | 0 (0) | 1 (5.0) | |
| Oxygen saturation in room air, %, median (IQR) | 96.00 (94.00, 98.00) | 97.00 (94.75, 98.00) | 94.00 (92.00, 96.00) | 0.005 |
| 6‐min walk test distance, m, mean (SD) | 352.56 (123.81) | 390.11 (101.55) | 282.47 (134.20) | 0.005 |
| Minimal oxygen saturation at 6‐min walk test, %, median (IQR) | 85.00 (81.50, 91.00) | 86.50 (82.75, 92.25) | 82.00 (71.50, 90.50) | 0.039 |
| Hemodynamic characteristics | ||||
| Mean pulmonary arterial pressure, mm Hg, mean (SD) | 55.8 (24.0) | 48.1 (21.3) | 71.7 (21.8) | 0.001 |
| Mean aortic pressure, mm Hg, mean (SD) | 63.1 (10.3) | 63.4 (8.5) | 62.6 (13.6) | 0.80 |
| Right arterial pressure, mm Hg, median (IQR) | 7.0 (5.0, 8.0) | 6.0 (5.0, 8.0) | 8.0 (7.3, 13.0) | 0.017 |
| Left atrial pressure, mm Hg, median (IQR) | 8.0 (7.0, 10.0) | 8.0 (6.0, 9.0) | 8.5 (7.0, 11.0) | 0.44 |
| Pulmonary artery oxygen saturation, %, median (IQR) | 69.5 (64.3, 75.6) | 73.0 (67.0, 82.0) | 65.0 (57.0, 71.0) | 0.019 |
| PVRI baseline, WU×m2, median (IQR) | 13.6 (9.3, 25.6) | 11.8 (6.5, 22.1) | 24.2 (13.5, 27.4) | 0.01 |
| PVRI with nitric oxide, WU×m2 median (IQR) | 12.0 (5.9, 19.5) | 10.3 (5.0, 15.2) | 15.8 (12.1, 26.2) | 0.03 |
| Echocardiographic characteristics | ||||
| Right heart dimensions | ||||
| Right atrial area, cm2 median (IQR) | 12.83 (9.75, 15.69) | 11.75 (9.28, 13.39) | 15.90 (11.10, 19.20) | 0.008 |
| Right atrial z score, median (IQR) | 2.92 (1.25, 5.11) | 1.77 (1.03, 3.21) | 5.31 (3.30, 7.21) | <0.001 |
| Tricuspid valve diameter, cm, mean (SD) | 2.75 (0.75) | 2.66 (0.68) | 2.95 (0.88) | 0.15 |
| Tricuspid valve diameter, z score, median (IQR) | 2.78 (0.76, 5.23) | 2.04 (0.32, 4.25) | 4.56 (2.31, 5.65) | 0.035 |
| RV internal diastolic diameter, cm, median (IQR) | 2.60 (1.85, 3.53) | 2.44 (1.60, 2.85) | 2.90 (2.28, 4.35) | 0.09 |
| RV internal systolic diameter, cm, median (IQR) | 2.40 (1.76, 3.70) | 2.20 (1.40, 2.60) | 3.45 (2.21, 4.48) | 0.036 |
| Pulmonary valve diameter, cm, mean (SD) | 2.15 (0.61) | 2.07 (0.61) | 2.31 (0.61) | 0.16 |
| Pulmonary valve diameter, z score, mean (SD) | 2.28 (2.66) | 1.72 (2.78) | 3.24 (2.17) | 0.04 |
| Right pulmonary artery dimension, cm mean (SD) | 1.44 (0.51) | 1.39 (0.52) | 1.53 (0.48) | 0.33 |
| Right pulmonary artery dimension, z score, mean (SD) | 1.72 (2.33) | 1.42 (2.47) | 2.24 (2.02) | 0.20 |
| Left pulmonary artery dimension, cm, mean (SD) | 1.45 (1.12, 1.97) | 1.44 (1.10, 1.95) | 1.50 (1.20, 2.00) | 0.56 |
| Left pulmonary artery dimension, z score, mean (SD) | 2.21 (1.02, 4.92) | 2.21 (0.56, 4.56) | 2.21 (1.11, 5.25) | 0.37 |
| Right ventricular function | ||||
| Semiquantitative RV function, I–IV, median (IQR) | 2.00 (1.00, 2.50) | 1.25 (1.00, 2.00) | 2.50 (2.00, 3.00) | <0.001 |
| Tricuspid annular plane systolic excursion, cm, mean (SD) | 1.59 (0.42) | 1.66 (0.45) | 1.47 (0.34) | 0.10 |
| Tricuspid annular plane systolic excursion, z score, mean (SD) | −2.77 (2.40) | −2.36 (2.52) | −3.56 (1.95) | 0.07 |
| RV dp.dt, median (IQR) | 1143 (964, 1620) | 1131 (966, 1403) | 1236 (924, 1629) | 0.75 |
| RV shortening fraction, %, mean (SD) | 39.5 (12.2) | 38.1 (11.7) | 42.3 (13.0) | 0.21 |
| PH severity parameters | ||||
| Tricuspid regurgitation Doppler velocity, m/s, mean (SD) | 4.27 (0.92) | 3.99 (0.85) | 4.79 (0.82) | 0.001 |
| Pulmonary acceleration time, ms, median (IQR) | 67.0 (51.0, 83.0) | 75.8 (59.5, 98.5) | 50.0 (47.0, 73.5) | 0.008 |
| Left heart dimensions | ||||
| Left atrial area, cm2, median (IQR) | 8.08 (3.48) | 8.09 (3.45) | 8.07 (3.62) | 0.98 |
| Left atrial z score, median (IQR) | −1.01 (−1.81, 0.55) | −1.02 (−1.75, 0.35) | −0.89 (−2.00, 0.76) | 0.90 |
| Mitral valve diameter, cm, mean (SD) | 1.98 (0.55) | 2.04 (0.56) | 1.84 (0.51) | 0.19 |
| Mitral valve diameter, z score, median (IQR) | −2.36 (2.46) | −2.18 (2.62) | −2.73 (2.09) | 0.42 |
| LV internal diastolic diameter, cm, median (IQR) | 31.72 (8.86) | 34.07 (8.38) | 27.26 (8.16) | 0.004 |
| LV internal systolic diameter, cm, median (IQR) | 19.24 (6.63) | 20.83 (5.63) | 16.21 (7.45) | 0.01 |
| Aortic valve diameter, cm, mean (SD) | 1.48 (0.38) | 1.47 (0.38) | 1.50 (0.40) | 0.75 |
| Aortic valve diameter, z score, mean (SD) | −1.92 (1.23) | −1.97 (1.33) | −1.81 (1.02) | 0.64 |
| Left ventricular function | ||||
| Ejection fraction, %, mean (SD) | 69.6 (14.8) | 67.8 (14.1) | 73.2 (16.0) | 0.19 |
| Left ventricular eccentricity indices | ||||
| Systolic LV eccentricity index, median, (IQR) | 1.9 (1.5, 2.5) | 1.70 (1.5, 2.2) | 2.4 (1.7, 3.4) | 0.014 |
| Diastolic LV eccentricity index, median, (IQR) | 1.5 (1.3, 1.8) | 1.3 (1.2, 1.6) | 1.8 (1.4, 2.0) | 0.001 |
| Tissue Doppler characteristics | ||||
| Tricuspid valve S‐wave, m/s, mean (SD) | 0.11 (0.02) | 0.11 (0.02) | 0.10 (0.03) | 0.29 |
| Tricuspid valve E‐wave, m/s, mean (SD) | 0.12 (0.04) | 0.13 (0.04) | 0.11 (0.05) | 0.15 |
| Tricuspid valve A‐wave, m/s, mean (SD) | 0.11 (0.03) | 0.11 (0.03) | 0.11 (0.02) | 0.75 |
| Interventricular septum S‐wave, m/s, median (IQR) | 0.06 (0.05, 0.07) | 0.06 (0.05, 0.07) | 0.06 (0.04, 0.07) | 0.12 |
| Interventricular septum E‐wave, m/s, median (IQR) | 0.07 (0.06, 0.10) | 0.08 (0.06, 0.10) | 0.06 (0.04, 0.08) | 0.02 |
| Interventricular septum A‐wave, m/s, median (IQR) | 0.06 (0.05, 0.07) | 0.06 (0.05, 0.06) | 0.06 (0.05, 0.07) | 0.70 |
| Mitral valve S‐wave, m/s, mean (SD) | 0.06 (0.05, 0.08) | 0.06 (0.06, 0.08) | 0.06 (0.05, 0.07) | 0.06 |
| Mitral valve E‐wave, m/s, mean (SD) | 0.11 (0.04) | 0.12 (0.04) | 0.09 (0.05) | 0.01 |
| Mitral valve A‐wave, m/s, mean (SD) | 0.05 (0.05, 0.07) | 0.05 (0.05, 0.06) | 0.06 (0.04, 0.08) | 0.34 |
P values refer to comparisons between patients with and without transplant‐free survival. dp/dt indicates delta pressure/delta time; IQR, interquartile range; IVS, interventricular septum; LV, left ventricular; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension; PVRI, pulmonary vascular resistance index; RV, right ventricular; WHO‐FC, World Health Organization functional classification; and WU, Wood units.