All patients | IPAH/FPAH | Group 1 PAH | Group 3 PH | p-value# | |||||
APAH non-CHD | APAH-CHD | ||||||||
All CHD | Shunt | ||||||||
Unrepaired/partial repair | Repaired | Never | |||||||
Patients N | 456 | 212 | 26 | 160 | 91 | 57 | 12 | 52 | |
ECG | 430 (94) | 148 (93) | 86 (95) | 50 (88) | 12 (100) | 49 (94) | 0.06 | ||
Chest radiograph | 406 (89) | 195 (92) | 24 (92) | 138 (86) | 77 (85) | 49 (86) | 12 (100) | 46 (88) | 0.05 |
Echocardiogram | 439 (96) | 205 (97) | 26 (100) | 154 (96) | 90 (99) | 52 (91) | 12 (100) | 50 (96) | 0.21 |
HC¶ | 445 (98) | 208 (98) | 24 (92) | 158 (99) | 91 (100) | 55 (96) | 12 (100) | 50 (96) | 0.40 |
Holter monitor | 94 (21) | 49 (23) | 5 (19) | 38 (24) | 18 (20) | 19 (33) | 1 (8) | 2 (4) | 0.001 |
Laboratory function tests | 411 (90) | 191 (90) | 23 (88) | 148 (93) | 85 (93) | 51 (89) | 12 (100) | 46 (88) | 0.42 |
6-min walk test | 175 (38) | 93 (44) | 11 (42) | 60 (38) | 38 (42) | 21 (37) | 1 (8) | 10 (19) | 0.003 |
Cardiopulmonary exercise test | 34 (7) | 16 (8) | 4 (15) | 7 (4) | 3 (3) | 4 (7) | 0 | 6 (12) | 0.17 |
Pulmonary function tests | 122 (27) | 67 (32) | 13 (50) | 31 (19) | 16 (18) | 13 (23) | 2 (17) | 9 (17) | 0.008 |
Overnight oxygen saturation and sleep recording | 129 (28) | 62 (29) | 9 (35) | 31 (19) | 13 (14) | 17 (30) | 1 (8) | 25 (48) | <0.001 |
Lung perfusion scintigraphy | 104 (23) | 69 (33) | 8 (31) | 16 (10) | 7 (8) | 9 (16) | 0 | 10 (19) | <0.001 |
Pulmonary angiography | 198 (43) | 102 (48) | 11 (42) | 49 (31) | 24 (26) | 22 (39) | 3 (25) | 33 (63) | <0.001 |
Chest computed tomography | 189 (41) | 102 (48) | 20 (77) | 36 (23) | 15 (16) | 17 (30) | 4 (33) | 27 (52) | <0.001 |
Magnetic resonance imaging | 42 (9) | 29 (14) | 4 (15) | 7 (4) | 4 (4) | 2 (4) | 1 (8) | 1 (2) | 0.001 |
Lung biopsy | 21 (5) | 8 (4) | 1 (4) | 9 (6) | 4 (4) | 5 (9) | 0 | 3 (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).