Table 1.
Demographic and clinical characteristics at diagnosis in all patients and in patients with confirmed pulmonary hypertension
| All patients | Patients with confirmed pulmonary hypertension
|
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|---|---|---|---|---|
| All | Incident | Prevalent | ||
| Patients | 456 (100%) | 362 (79%) | 102 (28%) | 260 (72%) |
| Female | 268 (59%) | 214 (59%) | 58 (57%) | 156 (60%) |
| Preterm | 63 (14%) | 47 (13%) | 16 (16%) | 31 (12%) |
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| ||||
| Age at diagnosis (years) | 7·1 (6·6–7·6) | 7·5 (7·0–8·1) | 8·5 (7·5–9·5) | 7·2 (6·5–7·8) |
|
| ||||
| Weight (kg) | 26·5 (24·7–28·4) | 28·0 (25·9–30·1) | 30·8 (26·7–35·0) | 26·8 (24·4–29·3) |
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| ||||
| Height (cm) | 117 (114–120) | 119 (116–123) | 124 (118–131) | 117 (113–121) |
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| ||||
| BMI (kg/m2) | 17·05 (16·63–17·48) | 17·21 (16·74–17·68) | 17·68 (16·67–18·69) | 17·02 (16·50–17·54) |
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| ||||
| BSA (m2) | 0·93 (0·88–0·97) | 0·94 (0·90–0·99) | 1·02 (0·93–1·11) | 0·91 (0·86–0·97) |
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| ||||
| Ethnicity | 454 (100%) | 362 (100%) | 102 (100%) | 260 (100) |
| White or Hispanic | 302 (67%) | 229 (63%) | 57 (56%) | 172 (66%) |
| Black | 14 (3%) | 8 (2%) | 5 (5%) | 3 (1%) |
| Asian | 107 (24%) | 99 (27%) | 33 (32%) | 66 (25%) |
| Other | 15 (3%) | 13 (4%) | 5 (5%) | 8 (3%) |
| Unknown | 16 (4%) | 13 (4%) | 2 (2%) | 11 (4%) |
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| ||||
| Time from onset symptoms to diagnosis (months) | 17 (15–20) | 17 (14–20) | 24 (16–31) | 15 (12–18) |
| Median (IQR) | 6 (2–19) | 6 (2–19) | 6 (3–38) | 5 (1–17) |
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| ||||
| Time from diagnosis to enrolment (months) | 24·0 (21·7–26·4) | 24·4 (21·8–27·1) | 0·7 (0·5–0·9) | 33·7 (30·7–36·7) |
| Median (range) | 13·7 (1·7–39·8) | 14·1 (2·3–39·8) | 0·2 (0·0–1·1) | 28·5 (12·6–48·9) |
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| ||||
| Group I* | 398 (87%) | 317 (88%) | 88 (86%) | 229 (88%) |
| IPAH or FPAH | 212 (53%) | 182 (57%) | 55 (63%) | 127 (55%) |
| APAH-congenital heart disease | 160 (40%) | 115 (36%) | 26 (30%) | 89 (39%) |
| Systemic-to-pulmonary shunt | 150 (38%) | 107 (34%) | 25 (28%) | 82 (36%) |
| Unrepaired | 91 (23%) | 61 (19%) | 14 (16%) | 47 (21%) |
| Repaired | 57 (14%) | 45 (14%) | 10 (11%) | 35 (15%) |
| Never shunt | 12 (3%) | 9 (3%) | 2 (2%) | 7 (3%) |
| Repaired left obstruction | 7 (2%) | 7 (2%) | 1 (1%) | 6 (3%) |
| APAH-connective tissue disease | 10 (3%) | 9 (3%) | 1 (1%) | 8 (3%) |
| APAH-chronic liver disease | 4 (1%) | 2 (1%) | 0 (0%) | 2 (1%) |
| APAH-HIV | 0 (0%) | 0 (0%) | 0 (0%) | 0 (0%) |
| APAH-drugs or toxins | 0 (0%) | 0 (0%) | 0 (0%) | 0 (0%) |
| APAH-HHT | 2 (1%) | 1 (<1%) | 1 (1%) | 0 (0%) |
| APAH-thyroid | 2 (1%) | 1 (<1%) | 0 (0%) | 1 (<1%) |
| APAH-other | 3 (1%) | 3 (1%) | 0 (0%) | 3 (1%) |
| PVOD or PCH | 6 (2%) | 6 (2%) | 4 (5%) | 2 (1%) |
| None of the above | 5 (1%) | 3 (1%) | 2 (2%) | 1 (<1%) |
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| Group 3* | 52 (11%) | 42 (12%) | 13 (13%) | 29 (11%) |
| Bronchopulmonary dysplasia | 17 (33%) | 11 (26%) | 3 (23%) | 8 (28%) |
| Interstititial lung disease | 12 (23%) | 10 (24%) | 4 (31%) | 6 (21%) |
| High altitude | 7 (13%) | 7 (17%) | 3 (23%) | 4 (14%) |
| Congenital diaphragmatic hernia | 6 (12%) | 4 (10%) | 1 (8%) | 3 (10%) |
| Congenital pulmonary hypoplasia | 7 (13%) | 5 (12%) | 2 (15%) | 3 (10%) |
| Disordered breathing or OSAS | 5 (10%) | 5 (12%) | 0 (0%) | 5 (17%) |
| Kyphoscoliosis | 2 (4%) | 2 (5%) | 0 (0%) | 2 (7%) |
| Other | 2 (4%) | 2 (5%) | 1 (8%) | 1 (3%) |
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| Groups 4 or 5* | 4 (1%) | 3 (1%) | 1 (1%) | 2 (1%) |
| WHO Functional Class | 449 (98%) | 362 (100%) | 102 (100%) | 260 (100%) |
| I | 54 (12%) | 45 (12%) | 11 (11%) | 34 (13%) |
| II | 212 (47%) | 185 (51%) | 52 (51%) | 133 (51%) |
| III | 146 (33%) | 108 (30%) | 28 (27%) | 80 (31%) |
| IV | 37 (8%) | 24 (7%) | 11 (11%) | 13 (5%) |
| 6 min walk test | 175 (38%) | 153 (42%) | 46 (45%) | 107 (41%) |
| Metres (mean [95% CI]) | 407 (389–426) | 417 (398–436) | 445 (414–476) | 405 (381–429) |
Data are n (%) or mean (95% CI) unless otherwise specified. Incident cases were those diagnosed within 3 months of enrolment. Prevalent cases were those diagnosed more than 3 months before enrolment. Two patients are missing for pulmonary hypertension classification because of missing data. Patients could be counted in more than one APAH disease category and in more than one associated disease category for group 3. BMI=body-mass index. BSA=body surface area. IPAH=idiopathic pulmonary arterial hypertension. FPAH=familial pulmonary arterial hypertension. APAH=pulmonary arterial hypertension associated with other disorders. HHT=hereditary haemorrhagic teleangiectasia. PVOD=pulmonary veno-occlusive disease. PCH=pulmonary capillary haemangiomatosis. OSAS=obstructive sleep apnoea syndrome.
Classified according to (3rd World Pulmonary Hypertension Symposium11).