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. Author manuscript; available in PMC: 2012 Aug 24.
Published in final edited form as: Lancet. 2012 Jan 11;379(9815):537–546. doi: 10.1016/S0140-6736(11)61621-8

Table 3.

Clinical symptoms at diagnosis, reported for 5% or more of all patients with confirmed pulmonary hypertension (PH confirmed), according to pulmonary hypertension groups and subgroups

All PH confirmed PH group 3 PH group 1
IPAH or FPAH APAH with congenital heart disease 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%)
Dyspnoea with exertion 235 (65%) 22 (52%) 121 (66%) 77 (67%) 48 (79%) 24 (53%) 5 (56%) 14 (70%)
Fatigue 149 (41%) 12 (29%) 82 (45%) 47 (41%) 29 (48%) 13 (29%) 5 (56%) 6 (30%)
Syncope 73 (20%) 3 (7%) 57 (31%) 10 (9%) 0 (0%) 8 (18%) 2 (22%) 3 (15%)
Cyanosis with exertion 64 (18%) 10 (24%) 24 (13%) 27 (23%) 22 (36%) 5 (11%) 0 (0%) 3 (15%)
Cough 48 (13%) 6 (14%) 32 (18%) 8 (7%) 1 (2%) 7 (16%) 0 (0%) 2 (10%)
Cyanosis with rest 44 (12%) 11 (26%) 7 (4%) 22 (19%) 15 (25%) 4 (9%) 3 (33%) 4 (20%)
Dyspnoea with rest 39 (11%) 12 (29%) 13 (7%) 11 (10%) 3 (5%) 6 (13%) 2 (22%) 3 (15%)
Chest pain or discomfort 39 (11%) 3 (7%) 24 (13%) 7 (6%) 4 (7%) 2 (4%) 1 (11%) 4 (20%)
Near-syncope 28 (8%) 1 (2%) 21 (12%) 5 (4%) 1 (2%) 4 (9%) 0 (0%) 1 (5%)
Dizziness 25 (7%) 2 (5%) 14 (8%) 6 (5%) 4 (7%) 2 (4%) 0 (0%) 2 (10%)
Palpitations 22 (6%) 0 (0%) 12 (7%) 6 (5%) 6 (10%) 0 (0%) 0 (0%) 3 (15%)
Pallor with exertion 17 (5%) 3 (7%) 8 (4%) 5 (4%) 5 (8%) 0 (0%) 0 (0%) 0 (0%)
Irritability 17 (5%) 5 (12%) 8 (4%) 3 (3%) 2 (3%) 1 (2%) 0 (0%) 1 (5%)

Data are number (%). 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. Full details are provided in the webappendix. APAH=pulmonary arterial hypertension associated with other disorders. IPAH=idiopathic pulmonary arterial hypertension. FPAH=familial pulmonary arterial hypertension. CHD=congenital heart disease.

*

Or partial repair.