Table 1.
Demographic, hemodynamic, and cardiac magnetic resonance data for all subjects
| Subject demographics (n = 17) | |
|---|---|
| Age | 11.0 years (range 0.25–23.0) |
| Male gender | 9/17 |
| BSA (m2) | 1.2 ± 0.5 |
| Pulmonary Hypertension Classificationa | |
| 1. Pulmonary arterial hypertension | 15 |
| 1.1 Idiopathic | 5 |
| 1.3 Drug and Toxin-induced (anthracycline) | 1 |
| 1.4.1 Connective tissue disorder (Overlap syndome) | 1 |
| 1.4.3 Portal hypertension | 1 |
| 1.4.4 Congenital heart disease | 6 |
| Atrial septal defect | 4 |
| Atrioventricular septal defect | 1 |
| Partial anomalous pulmonary venous return | 1 |
| 1.4.5 Pulmonary hypertension from schistosomiasis | 1 |
| 3. Pulmonary hypertension due to lung disease | 2 |
| World Health Organization Functional Classification | |
| WHO-FC I | 2 |
| WHO-FC II | 8 |
| WHO-FC III | 4 |
| WHO-FC IV | 1 |
| Pulmonary artery pressure | |
| Systolic pulmonary artery pressure (mmHg) | 54.5 (20.6) |
| Diastolic pulmonary artery pressure (mmHg) | 25.4 (11.7) |
| Mean pulmonary artery pressure (mmHg) | 35 (15) |
| Pulmonary capillary wedge pressure (mmHg) | 10.8 (3.2) |
| Pulmonary vascular resistance index (Woods unit x m2) | 8.5 (7.8) |
| Cardiac output indexed (L/min/m2) | 4.8 (1.5) |
| Right ventricular volume | |
| End-diastole (ml/m2) | 118.4 (51.1) |
| End-systole (ml/m2) | 70.9 (42.9) |
| Right ventricular ejection fraction (%) | 46.6 (9.7) |
| Right ventricular stroke volume indexed (ml/m2) | 54.4 (12.7) |
| Ea (mmHg/ml/m2) | 0.49 (0.26) |
| Emax (mmHg/ml/m2) | 0.56 (0.18) |
| VVCRs | 1.79 (0.34) |
| VVCRm | 1.29 (0.72) |
aPulmonary hypertension classification based on 5th World Symposium in Nice, France in 2013 from Simommeau G et al. Updated clinical classification of pulmonary hypertension. JACC 2013:62:D34-41