TABLE 2.
Diseases associated with paediatric pulmonary hypertension (PH) patients with World Symposium on Pulmonary Hypertension (WSPH) Groups 1, 1′ and 1″ as primary classification
| Group 1: Pulmonary arterial hypertension (PAH) | 602 (90.8) |
| 1.1 Idiopathic | 148 (22.3) |
| 1.2 Heritable | 40 (6.1) |
| 1.3 Drug and toxins induced | 2 (0.3) |
| 1.4 Associated with PAH | 410 (61.8) |
| 1.4.1 Connective tissue disease | 9 (1.4) |
| 1.4.2 HIV infection | 0 (0) |
| 1.4.3 Portal hypertension | 6 (0.9) |
| 1.4.4 Congenital heart disease# | 390 (58.8) |
| 1.4.4.1 Type | 312 (47.1) |
| 1.4.4.1.1 Simple pre-tricuspid shunts | 93 (14.0) |
| 1.4.4.1.1.1 Atrial septal defect | 78 (11.7) |
| 1.4.4.1.1.2 Total or partial unobstructed anomalous pulmonary venous return | 15 (2.3) |
| 1.4.4.1.2 Simple post-tricuspid shunts | 112 (16.9) |
| 1.4.4.1.2.1 Ventricular septal defect | 77 (11.6) |
| 1.4.4.1.2.2 Patent ductus arteriosus | 54 (8.1) |
| 1.4.4.1.3 Combined shunts | 35 (5.3) |
| 1.4.4.1.4 Complex congenital heart disease | 121 (18.2) |
| 1.4.4.1.4.1 Complete atrioventricular septal defect | 52 (7.8) |
| 1.4.4.1.4.2 Truncus arteriosus | 3 (0.5) |
| 1.4.4.1.4.3 Single ventricle physiology with unobstructed pulmonary blood flow | 20 (3.0) |
| 1.4.4.1.4.4 Transposition of the great arteries | 16 (2.4) |
| 1.4.4.2 Direction of shunt | 99 (14.9) |
| 1.4.4.2.1 Predominantly systemic-to-pulmonary | 51 (7.7) |
| 1.4.4.2.2 Predominantly pulmonary-to-systemic (Eisenmenger’s) | 27 (4.1) |
| 1.4.4.2.3 Bidirectional | 19 (2.9) |
| 1.4.4.3 Repair status | 170 (25.6) |
| 1.4.4.3.1 Unoperated | 43 (6.5) |
| 1.4.4.3.2 Partial repair | 18 (2.7) |
| 1.4.4.3.3 Complete repair | 109 (16.4) |
| 1.4.5 Schistosomiasis | 0 (0) |
| Group 1′: Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis | 11 (1.7) |
| Group 1″: Persistent PH of the newborn | 50 (7.6) |
Data are presented as n (%); all percentages are based on WSPH Groups 1, 1′ and 1″ (n=663) as the denominator. Category counts may be larger than the sum of counts for associated subclasses, due to the classification of disease for some patients only at a high-level subgroup.
#
: subclasses for congenital heart disease are not mutually exclusive, as more than one heart defect may be associated with a single patient.