Table 1.
Demographics and hemodynamics measurements
| PAH (n =42) | Control (n = 26) | p-value | |
|---|---|---|---|
| Age (yrs) | 14.6 ± 4.6 | 15.1 ± 3.2 | 0.6110 |
| sex – female (%) | 23 (54.7) | 13 (50.0) | 0.7956 |
| BMI | 19.6 ± 5.2 | 21.4 ± 3.1 | 0.0934 |
| BMI percentile | 42.3 ± 30.9 | 60.8 ± 22.7 | 0.0082 |
| BSA, (m2) | 1.40 ± 0.38 | 1.58 ± 0.27 | 0.0313 |
| IPAH, No(%) | 18 (42.8) | ||
| CHD-PAH | 22 (42.3) | ||
| ASD | 13 (30.9) | ||
| VSD | 5 (11.9) | ||
| PDA | 5 (11.9) | ||
| BPD | 2 (4.7) | ||
| Systolic BP (mmHg) | 106 ± 12 | 112 ± 9 | 0.1338 |
| Diastolic BP (mmHg) | 61 ± 7 | 64 ± 6 | 0.1683 |
| Pulse pressure (mmHg) | 45 ± 11 | 46 ± 10 | 0.8161 |
| Shunt present at CMR evaluation | 5 (11.9) | ||
| World Health Organization Functional Classification | |||
| WHO-FC I | 7 (16.6) | ||
| WHO-FC II | 17 (40.4) | ||
| WHO-FC III | 12 (28.5) | ||
| WHO-FC IV | 6 (14.2) | ||
| Cardiac Catheterization | |||
| mPAP (mmHg) | 44 ± 17 | ||
| PVRi (WU/m2) | 9 ± 7 | ||
| PAWP (mmHg) | 9 ± 2 | ||
| NT-proBNP (pg/mL) | 156 (73 - 445) | ||
| BNP (pg/mL) | 42 (15 - 106) | ||
| CMR Hemodynamics | |||
| LV end-diastolic volume (mL/m2)* | 93 ± 34 | 89 ± 18 | 0.8567 |
| LV end-systolic (mL/m2)* | 41 ± 17 | 39 ± 15 | 0.6614 |
| LV stroke volume (mL/m2)* | 48 ± 16 | 51 ± 9 | 0.3623 |
| LV ejection fraction (%) | 53 ± 10 | 58 ± 4 | 0.0231 |
| LV VVCR – ESV/SV | 0.99 ± 0.63 | 0.75 ± 0.17 | 0.0986 |
| Cardiac index (L/min/m2)* | 3.9 ± 0.9 | 3.8 ± 0.9 | 0.7198 |
| Heart rate (bpm) | 79 ± 16 | 72 ± 15 | 0.1254 |
| RV end-diastolic volume (mL/m2)* | 138 ± 73 | 92 ± 14 | 0.0005 |
| RV end-systolic volume (mL/m2)* | 84 ± 73 | 41 ± 9 | 0.0008 |
| RV stroke volume (mL)* | 53 ± 14 | 51 ± 10 | 0.5928 |
| RV ejection fraction (%) | 44 ± 14 | 58 ± 11 | 0.0012 |
| MPA diameter (cm/m2)* | 2.48 (2.09 - 2.93) | 1.61 (1.54 - 1.79) | < 0.0001 |
| Aortic diameter (cm/m2)* | 1.63 (1.44 - 1.94) | 1.60 (1.49 - 1.83) | 0.9792 |
| MPA/Aorta size ratio | 1.42 (1.23 - 1.67) | 1.07 (0.99 - 1.09) | < 0.0001 |
Data are expressed as averages ± SD or medians with inter-quantile range.
*
metrics are normalized by BSA, IPAH = idiopathic pulmonary arterial hypertension
CHD-PAH = pulmonary arterial hypertension associated with congenital heart disease
BPD = bronchopulmonary dysplasia, VVCR = ventricular-vascular coupling ratio, ASD = atrial septal defect
VSD = ventricular septal defect, PDA = patent ductus arteries