Table 4– Test performed at diagnosis by diagnostic groups
All patientsIPAH/FPAHGroup 1 PAHGroup 3 PHp-value#
APAH non-CHDAPAH-CHD
All CHDShunt
Unrepaired/partial repairRepairedNever
Patients N4562122616091571252
ECG430 (94)148 (93)86 (95)50 (88)12 (100)49 (94)0.06
Chest radiograph406 (89)195 (92)24 (92)138 (86)77 (85)49 (86)12 (100)46 (88)0.05
Echocardiogram439 (96)205 (97)26 (100)154 (96)90 (99)52 (91)12 (100)50 (96)0.21
HC445 (98)208 (98)24 (92)158 (99)91 (100)55 (96)12 (100)50 (96)0.40
Holter monitor94 (21)49 (23)5 (19)38 (24)18 (20)19 (33)1 (8)2 (4)0.001
Laboratory function tests411 (90)191 (90)23 (88)148 (93)85 (93)51 (89)12 (100)46 (88)0.42
6-min walk test175 (38)93 (44)11 (42)60 (38)38 (42)21 (37)1 (8)10 (19)0.003
Cardiopulmonary exercise test34 (7)16 (8)4 (15)7 (4)3 (3)4 (7)06 (12)0.17
Pulmonary function tests122 (27)67 (32)13 (50)31 (19)16 (18)13 (23)2 (17)9 (17)0.008
Overnight oxygen saturation and sleep recording129 (28)62 (29)9 (35)31 (19)13 (14)17 (30)1 (8)25 (48)<0.001
Lung perfusion scintigraphy104 (23)69 (33)8 (31)16 (10)7 (8)9 (16)010 (19)<0.001
Pulmonary angiography198 (43)102 (48)11 (42)49 (31)24 (26)22 (39)3 (25)33 (63)<0.001
Chest computed tomography189 (41)102 (48)20 (77)36 (23)15 (16)17 (30)4 (33)27 (52)<0.001
Magnetic resonance imaging42 (9)29 (14)4 (15)7 (4)4 (4)2 (4)1 (8)1 (2)0.001
Lung biopsy21 (5)8 (4)1 (4)9 (6)4 (4)5 (9)03 (6)0.62
  • Data are presented as n (%), unless otherwise stated. N: total number of patients; n: all patients who were confirmed as having undergone the procedure; PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; FPAH: familial PAH; APAH: associated PAH; CHD: congenital heart disease; PH: pulmonary hypertension; HC: heart catheterisation. For missing data, it was assumed that the procedure was not performed. #: Fisher's exact test to compare group 3 PH versus all-CHD versus IPAH/FPAH for diagnostic procedure being performed; : it was intended that all patients would undergo HC; however, 11 patients had indications on their case-report form that PH was not confirmed by HC, 10 patients provided no further HC data. Denominator for percentages is the number of subjects in the population in each subgroup (n).