Introduction
Methods
Study population
Data collection and outcome definition
Statistical analysis
Results
Patient cohort
All patients | The Netherlands | Singapore | |||||
---|---|---|---|---|---|---|---|
(n = 74) | (n = 45) | (n = 29) |
p value | ||||
Age (years) | 45 ± 14 | 47 ± 14 | 41 ± 14 | 0.11 | |||
Male sex, n (%) | 23 (31) | 14 (31) | 9 (31) | 0.99 | |||
Follow up (years) | 3.7 ± 2.4 | 3.3 ± 2.2 | 4.4 ± 2.8 | 0.06 | |||
Height (cm) | 163 ± 10 | 168 ± 8 | 158 ± 9 | <0.01
| |||
Weight (kg) | 61 ± 15 | 64 ± 14 | 57 ± 15 |
0.04
| |||
Body surface area (m2) | 1.7 ± 0.2 | 1.7 ± 0.2 | 1.6 ± 0.2 | <0.01
| |||
Eisenmenger syndrome, n (%) | 57 (77) | 32 (71) | 25 (86) | 0.13 | |||
Pretricuspid shunt, n (%) | 33 (45) | 16 (36) | 17 (59) |
0.05
| |||
Posttricuspid shunt, n (%) | 41 (55) | 29 (64) | 12 (41) | ||||
Shunt closed, n (%) | 4 (6) | 2 (5) | 2 (7) | 0.67 | |||
Exercise capacity | |||||||
NYHA functional class III, n (%) | 55 (74) | 32 (71) | 23 (79) | 0.36 | |||
peak VO2 (ml/min) | 792 ± 344 | 970 ± 357 | 660 ± 275 | <0.01
| |||
peak VO2 (% of predicted) | 41 ± 16 | 49 ± 18 | 35 ± 11 |
0.01
| |||
Baseline 6MWT (m) | 363 ± 136 | 395 ± 137 | 312 ± 121 |
0.01
| |||
Baseline 6MWT (% of predicted) | 62 ± 24 | 68 ± 25 | 53 ± 21 |
0.02
| |||
Echocardiography | |||||||
sPAP (mmHg) | 84 ± 35 | 76 ± 32 | 99 ± 37 |
0.01
| |||
Impaired RV function, n (%) | 17 (17) | 8 (23) | 9 (27) | 0.67 | |||
Impaired LV function, n (%) | 5 (5) | 4 (11) | 1 (3) | 0.19 | |||
PAH medication, n (%) | |||||||
ERA | 45 (61) | 45 (100) | 0 | <0.01
| |||
PDE-5 inhibitor | 29 (39) | 0 | 29 (100) | <0.01
| |||
Combination therapy initiated | 11 (15) | 8 (18) | 3 (10) | 0.24 | |||
Discontinued PAH therapy | 7 (10) | 7 (16) | 0 |
0.03
|
Exercise capacity
Clinical outcome
Determinants of change in six-minute walk distance
β | 95 % CI |
p value | ||||
---|---|---|---|---|---|---|
Female sex | 4.0 | − 14.1–22.2 | 0.66 | |||
Singaporean ethnicity | 7.4 | − 13.2–27.9 | 0.48 | |||
Age at start advanced therapy (per 5 year lower) | 4.5 | 0.8–8.1 |
0.017
| |||
Pretricuspid shunt | 0.7 | − 18.8–20.1 | 0.95 | |||
PDE-5 inhibitor treatment | 7.4 | − 13.2–27.9 | 0.48 |