Table 1.
Demographic and haemodynamic data at presentation and relation to survival time (to death or transplantation)
| No | Mean (SD) | Median | Range | Pearson correlation coefficients | ||
| r | p Value | |||||
| Survival time | ||||||
| Time to death or transplantation from diagnosis (years) | 21 | 4.6 (5.4) | 2.2 | 0.05–18.5 | NA | NA |
| Boys (years) | 12 | 7.4 | 6.2 | NA | NA | 0.20* |
| Girls (years) | 9 | 3.6 | 1.7 | NA | ||
| Asymptomatic patients (years) | 7 | 6.0 | 3.4 | NA | NA | 0.92* |
| Symptomatic patients (years) | 14 | 6.0 | 2.2 | NA | ||
| Data at presentation | ||||||
| Age (years) | 21 | 5.7 (6.1) | 3.8 | 0.01–18.5 | −0.19 | 0.41 |
| LA:Ao (mm) | 13 | 2.0 (0.4) | 2.1 | 1.4–2.5 | −0.61 | 0.03 |
| PCWP (mm Hg) | 16 | 18.8 (5.3) | 18 | 9–26 | −0.49 | 0.06 |
| Right atrial pressure (mm Hg) | 18 | 10.7 (4.3) | 9.5 | 5–21 | 0.27 | 0.29 |
| RVEDP (mm Hg) | 17 | 11.2 (4.7) | 11 | 2–20 | −0.48 | 0.05 |
| LVEDP (mm Hg) | 16 | 22.6 (7.3) | 25.5 | 10–34 | −0.51 | 0.04 |
| Pulmonary artery pressure (mm Hg) | 18 | 34.4 (14.1) | 28.5 | 21–60 | −0.05 | 0.84 |
| Cardiac index (l/min/m2) | 18 | 3.1 (1.0) | 2.9 | 1.9–5.1 | 0.07 | 0.78 |
| PVRI (index units) | 17 | 5.6 (3.8) | 4 | 1.8–15.0 | 0.39 | 0.12 |
*Log rank.
LA:Ao, left atrial to aortic root dimension ratio; LVEDP, left ventricle end diastolic pressure, NA, not applicable; PCWP, pulmonary capillary wedge pressure; PVRI, indexed pulmonary vascular resistance; RVEDP, right ventricular end diastolic pressure.