Table 1 Demographic and clinical characteristics by WHO FC
Characteristics | WHO FC I/II | WHO FC III |
|---|---|---|
(n = 23) | (n = 13) | |
Age, years | 62.5 (53.0–75.6) | 58.9 (52.9–64.3) |
Female, n (%) | 14 (60.9) | 8 (61.5) |
Time since diagnosis, years | 5.7 (1.6–8.9) | 2.0 (1.2–6.7) |
PH etiology, n (%) | ||
PAH | ||
Congenital heart disease | 5 (21.7) | 2 (15.4) |
Idiopathic | 4 (17.4) | – |
HIV–associated | 4 (17.4) | – |
Scleroderma | 3 (13.0) | 3 (23.1) |
Portopulmonary hypertension | 3 (13.0) | 2 (15.4) |
Eisenmenger syndrome | – | 3 (23.1) |
Drug-associated | 1 (4.4) | 1 (7.6) |
Connective tissue disease, not scleroderma | 1 (4.4) | – |
CTEPH | 2 (8.7) | 2 (15.4) |
Time undergoing treatment, years | 3.2 (1.3–5.9) | 1.7 (0.9–2.9) |
Type of treatment for PH, n (%) | ||
PDE-5 is | 18 (78.3) | 11 (84.6) |
ERA | 12 (52.2) | 7 (53.9) |
PC analogs and receptor agonists | 2 (8.7) | 2 (15.4) |
PH specific therapy, n (%) | ||
Monotherapy | 15 (65.2) | 6 (46.1) |
Double combination therapy | 6 (26.1) | 5 (38.5) |
Triple combination therapy | 2 (8.7) | 2 (15.4) |
Line of treatment, n (%) | ||
First-line | 17 (73.9) | 7 (53.8) |
Second line | 4 (17.4) | 4 (30.8) |
Third line | – | 2 (15.4) |
Fourth line | 2 (8.7) | – |
Number of concomitant drugs | 6 (3–8) | 7 (4–9) |
Number of comorbidities | 2 (1–3) | 2 (1–4) |
Diuretics, n (%) | ||
Yes | 15 (65.2) | 11 (84.6) |
No | 8 (34.8) | 2 (15.4) |
Oral anticoagulants, n (%) | ||
Yes | 6 (26.1) | 3 (23.1) |
No | 17 (73.9) | 10 (76.9) |
Oxygen therapy, n (%) | ||
Yes | 2 (8.7) | 3 (23.1) |
No | 21 (91.3) | 10 (76.9) |
At least 1 emergency visit in the last 12 months, n (%) | 7 (30.4) | 4 (30.8) |
Number of emergency visits in the last 12 months | 0 (0–1) | 0 (0–1) |
At least 1 hospital admission in the last 12 months, n (%) | 6 (26.1) | 5 (38.5) |
Number of hospital admissions in the last 12 months | 0 (0–1) | 0 (0–1) |
6MWD, meters | 447.4 (362.3–488.0) | 329.4 (296.9–390.0) |
SBP_Bas, mmHg | 110.0 (110.0–137.5) | 110.0 (90.0–125.0) |
SBP_Max, mmHg | 157.5 (130.0–180.0) | 135.0 (120.0–150.0) |
Borg dyspnea | 3 (2–5) | 7 (3–10) |
O2Sat_Bas, mmHg | 97.0 (96.0–98.0) | 95.0 (92.0–96.0) |
O2Sat_Min, mmHg | 92.0 (88.0–94.0) | 92.0 (84.0–92.0) |
NT-proBNP, pg/mL | 238.0 (122.0–592.0) | 487.0 (120.0–1348.0) |
mPAP, mmHg | 35.0 (23.0–40.0) | 34.5 (26.0–52.5) |
Cardiac output, L/min | 4.2 (3.0–5.2) | 4.1 (3.7–5.5) |
RAP, mmHg | 7.0 (5.0–12.0) | 5.5 (4.0–7.0) |
PVR, Wood units | 4.9 (2.8–6.6) | 4.3 (2.1–7.4) |