Table 1.
Demographic and clinical patient characteristics and treatment strategies
| Characteristic/treatment strategy | Postoperative PAH | Idiopathic PAH | P |
|---|---|---|---|
| Number of patients | 201 (55.2) | 163 (44.8) | N/A |
| Age at diagnosis, y | 18.7 (10.0, 31.5) | 7.3 (2.9, 18.3) | < 0.001 |
| Follow-up time, y | 4.2 ± 2.8 | 3.7 ± 2.6 | 0.069 |
| Female | 137 (68.2) | 92 (56.4) | 0.021 |
| Down syndrome | 5 (2.5) | 2 (1.2) | 0.384 |
| NYHA-FC III–IV | 28 (13.9) | 63 (38.7) | < 0.001 |
| 6MWD, m | 489.6 ± 86.9 | 423.8 ± 83.3 | < 0.001 |
| Room air SpO2, % | 97.4 ± 3.0 | 94.8 ± 5.8 | < 0.001 |
| BNP, pg/ml | 76.0 (35.8, 213.0) | 373.0 (93.0, 930.0) | 0.001 |
| SPAP, mm Hg | 71.5 ± 21.3 | 79.7 ± 20.0 | < 0.001 |
| RV/LV diameter ratio | 0.53 ± 0.18 | 0.87 ± 0.41 | < 0.001 |
| RHC parameters | 173 (86.1) | 83 (50.9) | N/A |
| mRAP, mm Hg | 9.0 ± 3.4 | 8.4 ± 3.4 | 0.243 |
| mPAP, mm Hg | 58.1 ± 22.4 | 61.7 ± 17.6 | 0.217 |
| PVRI, Wood units · m2 | 13.9 (9.2, 27.9) | 16.8 (10.0, 23.4) | 0.405 |
| Cardiac index, L/min/m2 | 3.2 ± 1.1 | 3.5 ± 1.5 | 0.119 |
| AVT | 11/173 (6.4) | 14/83 (16.9) | 0.008 |
| CCBs | 0/11 | 6/14 (42.9) | 0.030 |
| PAH Rx at last visit | 160 (79.6) | 123 (75.5) | 0.345 |
| ERA | 51 (31.9) | 47 (38.2) | 0.267 |
| PDE-5 inhibitor | 60 (37.5) | 21 (17.1) | < 0.001 |
| Prostacyclin | 9 (5.6) | 6 (4.9) | 0.781 |
| Combination therapy | 40 (25.0) | 49 (39.8) | 0.008 |
Values are n (%), median (interquartile range), or mean (±standard deviation).
AVT, acute pulmonary vasodilatation test; BNP, brain natriuretic peptide; CCB, calcium channel blockers; ERA, endothelin receptor antagonist; mPAP, mean pulmonary artery pressure; mRAP, mean right atrial pressure; N/A, not applicable; NYHA-FC, New York Heart Association functional class; PAH, pulmonary arterial hypertension; PDE, phosphodiesterase; PVRI, pulmonary vascular resistance index; RHC, right heart catheterization; RV/LV, right ventricle/left ventricle basal diameter ratio; Rx, treatment; SPAP, systolic pulmonary artery pressure; SpO2, percutaneous oxygen saturation; 6MWD, 6-minute walk distance.