Table 1 Baseline characteristics of newly registered patients using registration forms in 2012–2013 (for 2 years) (PoPH and I/H-PAH)
From: The clinical characteristics, treatment, and survival of portopulmonary hypertension in Japan
PoPH | I/H-PAH | p value | |
|---|---|---|---|
Number | 36 | 382 | |
Sex (F/M) | 20/16 | 219/163 | 0.8373 |
Age at diagnosis (years) | 50.4 ± 17.7 | 58.0 ± 20.8 | 0.0347 |
Age of onset (years) | 50.2 ± 20.3 | 55.1 ± 21.7 | 0.2544 |
Onset—First visit (months) | 3.6 ± 3.4 | 3.4 ± 3.1 | 0.7624 |
Onset—Diagnosis (years) | 2.3 ± 6.0 | 1.9 ± 6.7 | 0.6987 |
Hemodynamics | |||
sPAP (mmHg) | 72.1 ± 19.7 | 69.3 ± 20.1 | 0.4469 |
dPAP (mmHg) | 29.5 ± 11.1 | 28.6 ± 11.1 | 0.6790 |
mPAP (mmHg) | 46.1 ± 13.0 | 43.9 ± 13.4 | 0.3482 |
PAWP (mmHg) | 9.0 ± 3.1 | 9.8 ± 4.8 | 0.3321 |
RAP (mmHg) | 8.3 ± 5.5 | 8.2 ± 5.5 | 0.9373 |
PVR (dyne/s/cm−5) | 723.6 ± 483.5 | 830.1 ± 574.1 | 0.3011 |
CO (L/min) | 4.7 ± 1.7 | 3.9 ± 1.4 | 0.0086 |
CI (L/min/m2) | 2.8 ± 0.8 | 2.6 ± 1.0 | 0.2316 |
PvO2 (mmHg) | 42.2 ± 7.7 | 42.7 ± 13.8 | 0.9172 |
PvO2 (%) | 71.1 ± 11.6 | 70.5 ± 14.6 | 0.8313 |
6-min walk test | |||
6MWD (m) | 336.5 ± 15.9 | 263.7 ± 145.2 | 0.0368 |
Lowest SpO2 (%) | 86.7 ± 9.7 | 87.0 ± 7.8 | 0.8608 |
Blood exam | |||
BNP (pg/ml) | 600.6 ± 950.6 | 487.1 ± 593.2 | 0.3177 |
UA (mg/dl) | 6.3 ± 1.7 | 6.8 ± 2.4 | 0.2704 |
TRPG (mmHg) | 66.5 ± 19.9 | 66.5 ± 23.1 | 0.9861 |
History of right heart failure (±) | 14/20 | 149/229 | 0.8411 |
NYHA (1/2/3/4) | 2/14/16/4 | 11/115/185/61 | |
Modern PAH therapy (±) (%) | 19/17 [53] | 228/154 [60] | 0.4230 |
IV PGI2 (±) (%) | 1/35 [3] | 19/363 [5] | 0.5260 |
ERA (±) (%) | 9/27 [25] | 139/243 [36] | 0.1613 |
PDE5i (±) (%) | 14/22 [39] | 151/231 [40] | 0.9401 |
Combination therapy (±) (%) | 4/32 [11] | 75/307 [20] | 0.1856 |