Table 2.
Demographic and clinical characteristics at diagnosis in patients with confirmed pulmonary hypertension (PH confirmed) diagnosis according to pulmonary hypertension groups and subgroups
| All PH confirmed | PH group 3 | PH group 1
|
||||||
|---|---|---|---|---|---|---|---|---|
| IPAH or FPAH | APAH with CHD
|
APAH excluding APAH with CHD | ||||||
| All CHD | Unrepaired shunt* | Repaired shunt | Never shunt | |||||
| Patients | 362 (100%) | 42 (100%) | 182 (100%) | 115 (100%) | 61 (100%) | 45 (100%) | 9 (100%) | 20 (100%) |
| Female | 214 (59%) | 26 (62%) | 109 (60%) | 66 (57%) | 38 (62%) | 25 (56%) | 3 (33%) | 11 (55%) |
| Preterm | 47 (13%) | 18 (43%) | 14 (8%) | 11 (10%) | 5 (8%) | 6 (13%) | 0 (0%%) | 4 (20%) |
|
| ||||||||
| Age at diagnosis (years) (mean [95% CI]) | 7·5 (7·0–8·1) | 5·5 (3·7–7·3) | 7·6 (6·9–8·3) | 7·7 (6·7–8·8) | 8·4 (6·9–9·9) | 7·4 (5·8–8·9) | 4·8 (0·6–9·0) | 10·0 (7·5–12·4) |
|
| ||||||||
| Incident patients | 102 (28%) | 13 (31%) | 55 (30%) | 26 (23%) | 14 (23%) | 10 (22%) | 2 (22%) | 7 (35%) |
|
| ||||||||
| Ethnicity | 362 (100) | 42 (100) | 182 (100) | 115 (100) | 61 (100) | 45 (100) | 9 (100) | 20 (100) |
| White or Hispanic | 229 (63%) | 35 (83%) | 118 (65%) | 65 (57%) | 35 (57%) | 28 (62%) | 2 (22%) | 8 (40%) |
| Black | 8 (2%) | 0 (0%) | 5 (3%) | 2 (2%) | 2 (3%) | 0 (0%) | 0 (0%) | 1 (5%) |
| Asian | 99 (27%) | 3 (7%) | 48 (26%) | 40 (35%) | 23 (38%) | 12 (27%) | 5 (56%) | 8 (40%) |
| Other | 13 (4%) | 2 (5%) | 5 (3%) | 3 (3%) | 1 (2%) | 2 (4%) | 0 (0%) | 3 (15%) |
| Unknown | 13 (4%) | 2 (5%) | 6 (3%) | 5 (4%) | 0 | 3 (7%) | 2 (22%) | 0 (0%) |
|
| ||||||||
| Time from onset symptoms to diagnosis (months) (mean [95% CI]) | 17 (14–20) | 16 (7–25) | 15 (11–19) | 24 (17–32) | 30 (16–44) | 19 (11–26) | 22 (4–40) | 5 (1–8) |
| Median (IQR) | 6 (2–19) | 4 (1–20) | 5 (1–17) | 9 (4–29) | 10 (3–28) | 8 (4–25) | 9 (7–45) | 3 (1–4) |
|
| ||||||||
| Time from diagnosis to enrolment (months) (mean [95% CI]) | 24 (22–27) | 25 (18–32) | 25 (21–29) | 24 (19–28) | 23 (16–29) | 25 (18–32) | 24 (3–46) | 16 (6–27) |
| Median (IQR) | 14 (2–40) | 22 (2–40) | 15 (1–43) | 14 (4–41) | 13 (4–36) | 19 (6–45) | 20 (4–26) | 8 (1–23) |
|
| ||||||||
| WHO functional class | 362 (100%) | 42 (100%) | 182 (100%) | 115 (100%) | 61 (100%) | 45 (100%) | 9 (100%) | 20 (100%) |
| I | 45 (12%) | 8 (19%) | 28 (15%) | 8 (7%) | 2 (3%) | 4 (9%) | 2 (22%) | 1 (5%) |
| II | 185 (51%) | 20 (48%) | 84 (46%) | 72 (63%) | 40 (66%) | 28 (62%) | 4 (44%) | 8 (40%) |
| III | 108 (30%) | 13 (31%) | 55 (30%) | 31 (27%) | 18 (30%) | 13 (29%) | 0 (0%) | 7 (35%) |
| IV | 24 (7%) | 1 (2%) | 15 (8%) | 4 (3%) | 1 (2%) | 0 (0%) | 3 (33%) | 4 (20%) |
|
| ||||||||
| 6 min walk test | 153 (42%) | 10 (24%) | 83 (46%) | 50 (43%) | 30 (49%) | 19 (42%) | 1 (11%) | 9 (45%) |
| Metres (mean [95% CI]) | 417 (398–436) | 466 (352–580) | 407 (379–434) | 422 (393–452) | 420 (385–456) | 429 (370–488) | 355 (NC) | 427 (330–525) |
Patients from pulmonary hypertension groups 4 and 5 (n=3), included in all patients with confirmed pulmonary hypertension, are not depicted separately in this table. APAH=pulmonary arterial hypertension associated with other disorders. IPAH=idiopathic pulmonary arterial hypertension. FPAH=familial pulmonary arterial hypertension. CHD=congenital heart disease. NC=not calculated.
*
Or partial repair.