Table 2.
Clinical characteristics of the study population
| All patients (n=43) | I-PAH (n=8) | CHD-PAH (n=35) | |
|---|---|---|---|
| Age, years (mean±SD) | 37±12 | 42±9 | 36±12 |
| Age at diagnosis, years (mean±SD) | 26±12 | 35±10 | 24±11 |
| Gender [n (%)] | |||
| Male | 21 (49) | 2 (25) | 19 (54) |
| Female | 22 (49) | 6 (75) | 16 (46) |
| WHO functional class [n (%)] | |||
| I | - | - | - |
| II | 18 (42) | 2 (25) | 16 (44) |
| III | 23 (53) | 4 (50) | 19 (56) |
| IV | 2 (5) | 2 (25) | - |
| Family history of PAH [n (%)] | 1 (2.3) | 1 (12.5) | 0 |
| Echocardiographic findings (mean±SD) | |||
| LV end-diastolic diameter, mm | 41±8 | 36±7 | 42±7 |
| LV end-systolic diameter, mm | 26±7 | 22±5 | 27±8 |
| RV end-diastolic diameter, mm | 36±10 | 36±11 | 35±10 |
| PA diameter, mm | 33±7 | 32±9 | 34±7 |
| Tricuspid regurgitation velocity | 4.1±0.7 | 3.7±1 | 4.2±0.5 |
| Systolic PA pressure, mm Hg | 78±21 | 74±24 | 79±20 |
| Mean PA pressure*, mm Hg | 70±20 | 59±17 | 72±20 |
*
measured by right heart catheterization
CHD-PAH - PAH associated with CHD; I-PAH - idiopathic PAH; LV - left ventricular; RV - right ventricular; PA - pulmonary artery; PAH - pulmonary arterial hypertension