Table 1.
Baseline characteristics
| Controls (n = 18) | PS (n = 16) | iPAH (n = 18) | P‐value PS versus iPAH | |
|---|---|---|---|---|
| Age (Yrs.)* | 11.2 ± 5.0 | 10.3 ± 4.7 | 11.5 ± 5.6 | 0.736 |
| BSA (m2)* | 1.31 ± 0.44 | 1.23 ± 0.48 | 1.16 ± 0.41 | 0.547 |
| Male sex (%) | 10 (56%) | 9 (56%) | 9 (50%) | 0.716 |
| Heart rate (bpm) | 70 ± 13 | 81 ± 18 | 89 ± 22# | 0.261 |
| QRS ECG (msec) | – | 84 ± 11 | 99 ± 18 | 0.005 |
| RVSP (mmHg) | – | 75.4 ± 18.9 | 96.8 ± 25.4 | 0.011 |
| RV:LV pressure | – | 0.71 [0.41–1.57] | 1.10 [0.46–1.50] | 0.007 |
|
RVDd (mm) Z‐score |
18.5 ± 4.4 0.24 ± 1.02 |
20.3 ± 5.2 1.2 ± 1.56 |
34.4 ± 9.9*
4.82 ± 1.99* |
<0.001 <0.001 |
| RVDbas (mm) | 33.5 ± 5.0 | 34.4 ± 7.4 | 48.3 ± 10.0* | <0.001 |
| RVDmaj (mm) | 60.4 ± 10.9 | 56.4 ± 11.2 | 66.6 ± 12.3 | 0.010 |
| FAC (%) | 47.2 [39.2–53.7] | 42.5 [36.7–68.1] | 18.5 [8.1–34.3]* | <0.001 |
Patients were compared to controls using ANOVA with post hoc Dunnet's test # P = 0.002 and *P < 0.001. PS and iPAH were compared using independent Student T‐test.
PS, pulmonary stenosis; iPAH, idiopathic pulmonary arterial hypertension; Yrs., years; BSA, body surface area; bpm, beats per minute; RVSP, right ventricular systolic pressure; LV, left ventricular; RVDd, right ventricular diastolic dimension parasternal long‐axis; RVDbas, basal AP4CH‐dimension; RVmajor, long AP4CH‐dimension; FAC, fractional area change.