Summary of patient demographics and baseline characteristics
| Idiopathic PAH (n = 20) | Secondary PH (n = 20) | |
|---|---|---|
| *One junctional rhythm, one paced; †five superior axis, one paced; ‡n = 18; §three patients had systemic hypertension associated with connective tissue disease; ¶six aspirin, six warfarin. | ||
| PAH, pulmonary arterial hypertension; PAP, pulmonary arterial pressure; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; RV, right ventricular; SAP, systemic arterial pressure; WHO, World Health Organization. | ||
| Age at treatment (years) | ||
| Mean | 8.03 | 8.3 |
| Range | 1.2–17 | 0.6–16 |
| Boys:girls | 5:15 | 9:11 |
| Ethnicity | ||
| White | 19 | 18 |
| Afro-Caribbean | 0 | 2 |
| Asian | 1 | 0 |
| Weight (kg) | 27.5 (8–70) | 23.9 (6.8–59.3) |
| WHO functional class | ||
| II | 1 | 0 |
| III | 11 | 13 |
| IV | 8 | 7 |
| ECG findings | ||
| Sinus rhythm | 20 | 18* |
| Right axis deviation | 20 | 14† |
| RV hypertrophy with strain | 15 | 9 |
| Echocardiography | ||
| Tricuspid jet velocity (m/s) | 4.6 | 4.14 |
| Poor RV function | 12 | 5 |
| Atrial communication | 11 | 5 |
| Cardiac catheterisation | ||
| PAP>SAP | 9 | 3 |
| PAP = SAP | 7 | 7 |
| PAP<SAP | 4 | 8 |
| PVR (units⋅m2) | 21.7 (5.6–42.8) | 18.0 (4.9–49)‡ |
| Treatment at start of study | ||
| Nocturnal oxygen | 20 | 20 |
| Diuretics | 5 | 6 |
| Nifedipine | 0 | 4§ |
| Sildenafil | 2 | 4 |
| Epoprostenol | 7 | 2 |
| Anticoagulation | 12¶ | 2 |
| Duration of treatment (months) | 13.1 (3–24) | 12.2 (2–22) |