Tables
- Table 1– Diagnostic tests aiming at diagnosing/confirming pulmonary hypertension
Incident patients Prevalent patients All patients p-value# Patients N 135 321 456 ECG 131 (97) 299 (93) 430 (94) 0.11 Abnormal 116 (89) 269 (90) 385 (90) Chest radiograph 126 (93) 280 (87) 406 (89) 0.07 Abnormal 103 (82) 222 (80) 325 (80) Echocardiogram 132 (98) 307 (96) 439 (96) 0.45 Abnormal 132 (100) 304 (99) 436 (99) All three tests normal¶ 0 0 0 Two tests normal¶ 6 (4) 9 (3) 15 (3) One test normal¶ 23 (17) 58 (18) 81 (18) Heart catheterisation+ 131 (97) 314 (98) 445 (98) 0.74 Data are presented as n (%), unless otherwise stated. N: total number of patients; n: all patients who were confirmed to have undergone the procedure. Denominator for percentages is N (test performed) and number of patients with test within test (abnormal). #: Fisher's exact test to compare incident versus prevalent for diagnostic procedure being performed. ¶: ECG, echocardiogram and chest radiograph; +: it was intended that all patients would undergo heart catheterisation; however, 11 patients had indications on their case-report form that pulmonary hypertension was not confirmed by heart catheterisation, 10 patients provided no further heart catheterisation data.
- Table 2– Diagnostic tests aimed at excluding/diagnosing associate conditions
Incident patients Prevalent patients All patients p-value# Patients N 135 321 456 Holter monitor 33 (24) 61 (19) 94 (21) 0.21 Abnormal 26 (79) 27 (44) 53 (56) Pulmonary function tests 40 (30) 82 (26) 122 (27) 0.42 Total lung capacity L 3.87 (3.21–4.54) 3.77 (2.92–4.61) 3.81 (3.27–4.35) Patients 11 16 27 Total lung capacity % predicted 85.9 (79.4–92.3) 86.8 (81.4–92.2) 86.6 (82.4–90.7) Patients 14 37 51 Overnight oxygen saturation and sleep recording 29 (21) 100 (31) 129 (28) 0.040 Oxygen measured in room air 22 (16) 66 (21) 88 (19) Abnormal 11 (50) 18 (27) 29 (33) Measured in supplemental oxygen 9 (7) 33 (10) 42 (9) Abnormal 4 (44) 9 (27) 13 (31) Complete sleep recording 11 (8) 57 (18) 68 (15) Abnormal 7 (64) 30 (53) 37 (54) Lung perfusion scintigraphy 26 (19) 78 (24) 104 (23) 0.27 Abnormal 14 (54) 29 (37) 43 (41) Selective pulmonary artery angiography 49 (36) 149 (46) 198 (43) 0.048 Abnormal 28 (57) 58 (39) 86 (43) Chest computed tomography 58 (43) 131 (41) 189 (41) 0.75 Abnormal 47 (81) 93 (71) 140 (74) Magnetic resonance imaging 12 (9) 30 (9) 42 (9) >0.99 Abnormal 9 (75) 26 (87) 35 (83) Lung biopsy 4 (3) 16 (5) 20 (4) 0.34 Abnormal 4 (100) 15 (88) 19 (90) Haemoglobin g·L−1 134.6 (130.7–138.4) 137.4 (134.4–140.4) 136.5 (134.1–138.8) Patients 130 (96) 273 (85) 403 (88) Platelet count 109·L−1 243.4 (226.2–260.5) 248.6 (236.9–260.3) 246.8 (237.2–256.4) Patients 125 (93) 241 (75) 366 (80) Antinuclear antibody 74 (55) 152 (47) 226 (50) Yes 15 (20) 66 (43) 81 (36) Anticardiolipin IgG 57 (42) 111 (35) 168 (37) Positive or intermediate 2 (4) 7 (6) 9 (5) Anticardiolipin IgM 57 (42) 110 (34) 167 (37) Positive or intermediate 1 (2) 3 (3) 4 (2) Lupus anticoagulant 57 (42) 110 (34) 167 (37) Elevated 45 (79) 65 (59) 110 (66) Coagulation disorder 97 (72) 195 (61) 292 (64) Yes 4 (4) 9 (5) 13 (4) Thyroid function tests 68 (50) 136 (42) 204 (45) Aspartate transaminase¶ 111 (82) 237 (74) 348 (76) Elevated 7 (6) 20 (8) 27 (8) Alanine transaminase+ 112 (83) 240 (75) 352 (77) Elevated 4 (4) 14 (6) 18 (5) γ-glutamyl transpeptidase 86 (64) 175 (55) 261 (57) Elevated 3 (3) 10 (6) 13 (5) Alkaline phosphatase 94 (70) 223 (69) 317 (70) Elevated 6 (6) 11 (5) 17 (5) Total bilirubin, 106 (79) 235 (73) 341 (75) Elevated 3 (3) 14 (6) 17 (5) Direct bilirubin 89 (66) 186 (58) 275 (60) Elevated 3 (3) 8 (4) 11 (4) Data are presented as n (%), mean (95% CI) or n, unless otherwise stated. N: total number of patients; n: all patients who were confirmed as having undergone the procedure; Ig: immunoglobulin. Denominator for percentages is N for the test performed rows and the number of subjects with the test performed for the results rows. #: Fisher's exact test to compare incident versus prevalent for diagnostic procedure being performed; ¶: also known as serum glutamic-oxaloacetic transaminase; +: also known as serum glutamic-pyruvic transaminase.
- Table 3– Assessment of severity
Incident patients Prevalent patients All patients p-value# Patients N 135 321 456 6-min walk test 51 (38) 124 (39) 175 (38) 0.92 6-min walking distance m 438.9 (408.8–469.0) 394.2 (371.3-417.0) 407.2 (388.7–425.7) Cardiopulmonary exercise testing Patients 5 (4) 29 (9) 34 (7) 0.05 Peak V′O2, n 5 28 33 % predicted 58.0 (28.1–87.9) 50.6 (42.6–58.7) 51.8 (44.3-59.2) B-type natriuretic peptide ng·L−1 298.7 (115.7–481.7) 337.3 (177.1–497.6) 325.0 (203.1–446.9) Patients 31 (23) 66 (21) 97 (21) N-terminal b-type natriuretic peptide ng·L−1 4205 (953–7457) 4417 (2702–6131) 4343 (2788–5898) Patients 33 (24) 62 (19) 95 (21) Uric acid μmol·L−1 317.1 (288.3–345.9) 317.6 (297.0–338.3) 317.4 (300.8–334.0) Patients 61 (45) 108 (34) 169 (37) Troponin 26 (19) 38 (12) 64 (14) Elevated 3 (12) 3 (8) 6 (9) C-reactive protein mg·L−1 7.8 (1.6–14.1) 5.2 (3.9–6.5) 6.1 (3.8–8.3) Patients 68 (50) 137 (43) 205 (45) Data are presented as n (%) or mean (95% CI), unless otherwise state. N: total number of patients; n: all patients who were confirmed as having undergone the procedure. Denominator for percentages was N for the test-performed rows and the number of subjects with the test performed for the results rows.
- Table 4– Test performed at diagnosis by diagnostic groups
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).
- Table 5– Test performed at diagnosis by age groups
Patient age All patients p-value# 3≤24 months 2–6 years 7–11 years 12–18 years Patients¶ N 94 134 108 118 456 ECG 90 (96) 127 (95) 102 (94) 111 (94) 430 (94) 0.42 Chest radiograph 87 (93) 115 (86) 94 (87) 110 (93) 406 (89) 0.02 Echocardiogram 88 (94) 129 (96) 106 (98) 116 (98) 439 (96) 0.58 HC+ 91 (97) 132 (99) 103 (95) 117 (99) 445 (98) 0.25 Holter monitor 9 (10) 26 (19) 21 (19) 38 (32) 94 (21) 0.001 Laboratory function tests 78 (83) 117 (87) 103 (95) 113 (96) 411 (90) 0.002 6-min walk test§ 0 32 (24) 59 (55) 84 (71) 175 (38) <0.001 Cardiopulmonary exercise test 0 3 (2) 12 (11) 19 (16) 34 (7) <0.001 Pulmonary function tests 2 (2) 16 (12) 40 (37) 64 (54) 122 (27) <0.001 Overnight oxygen saturation and sleep recording 31 (33) 45 (34) 23 (21) 30 (25) 129 (28) 0.10 Lung perfusion scintigraphy 11 (12) 27 (20) 28 (26) 38 (32) 104 (23) 0.004 Pulmonary angiography 42 (45) 61 (46) 45 (42) 50 (42) 198 (43) 0.89 Chest computed tomography 36 (38) 53 (40) 46 (43) 54 (46) 189 (41) 0.70 Magnetic resonance imaging 1 (1) 4 (3) 13 (12) 24 (20) 42 (9) <0.001 Lung biopsy 5 (5) 8 (6) 4 (4) 4 (3) 21 (5) 0.75 Data are presented as n (%) unless otherwise stated. N: total number of patients; n: all patients who were confirmed as having undergone the procedure. HC: heart catheterisation. #: Fisher's exact test to compare across the age groups for diagnostic procedure being performed; ¶: the exact date of birth could not be obtained for two patients; however, both were in the paediatric age group and included in the general analysis and do not appear in this table; + it was intended that all patients would undergo HC; however, 11 patients had indications on their case-report form that pulmonary hypertension was not confirmed by HC, 10 patients provided no further HC data. §: nine of these patients (2–6 years n = 2; 7–11 years n = 1; 12–8 years n = 6) have Down syndrome or other chromosomal abnormalities. The denominator for percentages was N.
- Table 6– Haemodynamic parameters
Incident patients Prevalent patients All patients Patients N 135 321 456 Mean pulmonary arterial pressure mmHg 57.7 (54.0–61.4) 57.2 (55.2–59.2) 57.4 (55.6–59.1) Patients 126 307 433 Pulmonary vascular resistance index Wood units·m−2 15.9 (13.7–18.1) 15.7 (14.5–16.9) 15.8 (14.7–16.8) Patients 99 270 369 Cardiac index L·min−1·m−2 3.6 (3.3–4.0) 4.1 (3.5–4.7) 4.0 (3.5–4.5) Patients 96 269 365 Pulmonary vascular resistance index/systemic vascular resistance index 0.8 (0.7–0.9) 0.9 (0.8–0.9) 0.8 (0.8–0.9) Patients 92 260 352 SvO2 % 64.0 (61.2–66.7) 66.5 (65.0–67.9) 65.7 (64.4–67.0) Patients 79 188 267 Pulmonary capillary wedge pressure mmHg 8.8 (8.3–9.2) 8.4 (8.1–8.7) 8.5 (8.3–8.8) Patients 127 300 427 Mean right atrial pressure mmHg 7.2 (6.5–8.0) 7.0 (6.6–7.5) 7.1 (6.7–7.5) Patients 125 289 414 Data are shown as mean (95% CI) or n (%), unless otherwise stated. SvO2: mixed venous oxygen saturation.
- Table 7– Heart catheterisation (HC) complications at diagnosis
Incident patients Prevalent patients All patients p-value# Patients N 244 324 568 HC¶ 238 (98) 316 (98) 554 (98) Patients with significant complications during/after HC 15 (6) 22 (7) 37 (7) 0.86 Hypotension requiring intervention 8 (3) 9 (3) 17 (3) Inotropic support required 7 (3) 7 (2) 14 (3) PH crisis 7 (3) 3 (<1) 10 (2) Arrhythmia requiring intervention 1 (<1) 3 (<1) 4 (<1) Unexpected ICU admission after HC 4 (2) 3 (<1) 7 (1) Cardiac arrest 4 (2) 1 (<1) 5 (<1) Other 0 5 (2) 5 (<1) Pulmonary haemorrhage 0 1 (<1) 1 (<1) Death+/stroke/haemothorax/pneumothorax/pericardial effusion/cardiac perforation/bleeding requiring transfusion 0 0 0 Peri-HC complications recorded as reason for death§ 2 (<1) 0 2 (<1) Data are presented as n (%) unless otherwise stated. ICU: intensive care unit. #: Fisher's exact test to compare incident versus prevalent. ¶: it was intended that all patients would undergo HC; however, 11 patients had indications on their case-report form that pulmonary hypertension (PH) was not confirmed by HC, 10 patients provided no further HC data; +: death during procedure or within 24 h (recorded as a complication in the case report form; §: deaths relating to HC at diagnosis are presented in the table, of note, one of these patients did not undergo HC and died whilst being anesthetised in preparation for the HC. Three further patients (all prevalent) had “peri-HC complications” recorded as the reason for death following follow-up HCs. For number of patients with HC and number of patients with peri-HC complications recorded as reason for death the denominator for percentages is N. All other percentages were based on the number of patients undergoing HC as the denominator (n).
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