Table 1.
Cohort characteristics.
| CHD-PAH (n = 13) | |||
|---|---|---|---|
| Age (years) | 30.2 ± 2.2 | EF (%) | 63.2 ± 2.0 |
| Female, n (%) | 7 (54) | SV (mL) | 61.1 ± 5.1 |
| BMI (kg/m2) | 18.9 ± 0.8 | ||
| PAWP (mmHg) | 9.2 ± 0.6 | Type, n (%) | |
| mPAP (mmHg) | 70.9 ± 2.1 | ASD | 2 (15) |
| PVR (Woods units) | 12.7 ± 1.0 | VSD | 9 (69) |
| mRAP (mmHg) | 6.2 ± 0.8 | PDA | 1 (8) |
| 6MWD (m) | 468.0 ± 16.7 | ASD + VSD | 1 (8) |
| NT-proBNP (pg/mL) | 230.9 ± 55.4 | Drug therapy, n (%) | |
| Creatinine (μmol/L) | 70.6 ± 3.3 | PDE5 inhibitors | 10 (77) |
| TAPSE (mm) | 19.3 ± 1.0 | ERA | 5 (38) |
| Qp (L/min) | 5.0 ± 0.4 | Prostanoids | 9 (69) |
| Qs (L/min) | 3.5 ± 0.2 | CCB | 0 (0) |
BMI body mass index, PAWP pulmonary arterial wedge pressure, mPAP mean pulmonary arterial pressure, PVR pulmonary vascular resistance, mRAP mean right atrial pressure, 6MWD 6-min walking distance, NT-proBNP N-terminal pro-B-type natriuretic peptide, TAPSE tricuspid annular plane systolic excursion, Qp pulmonary circulation volume, Qs systemic circulation volume, EF ejection fraction, SV stroke volume, ASD atrial septal defect, VSD ventricular septal defect, PDA patent ductus arteriosus, ERA endothelin receptor antagonist, PDE5 phosphodiesterase type 5, CCB calcium channel blocker.