From: The clinical characteristics, treatment, and survival of portopulmonary hypertension in Japan
PoPH | I/H-PAH | p value | |
---|---|---|---|
Number | 11 | 39 | |
Sex (F/M) | 8/3 | 28/11 | 0.9514 |
Age (years) | 45.8 ± 11.8 | 49.1 ± 18.2 | 0.5812 |
Diagnosis (1999–2010/2011–2017) | 10/1 | 17/22 | 0.0029 |
Aetiology of PoPH (n (%)) | |||
PoPH with cirrhosis | 6 (55) | ||
Child–Pugh (A, B, C) | 5, 0, 1 | ||
HCV | 4 (36) | ||
Primary biliary cirrhosis and Autoimmune | 1 (9) | ||
Biliary atresia | 1 (9) | ||
PoPH without cirrhosis | 5 (45) | ||
Cryptogenic | 2 (18) | ||
Congenital portosystemic venous shunt | 3 (27) | ||
Haemodynamics | |||
mPAP (mmHg) | 48.9 ± 10.5 | 46.5 ± 14.4 | 0.6065 |
PVR (dyne/s/cm−5) | 772.5 ± 617.4 | 820.5 ± 443.7 | 0.7730 |
PAWP (mmHg) | 7.2 ± 3.1 | 7.8 ± 3.2 | 0.5489 |
RAP (mmHg) | 7.1 ± 6.0 | 5.4 ± 3.5 | 0.2348 |
CO (L/min) | 5.29 ± 1.6 | 4.05 ± 0.9 | 0.0016 |
CI (L/min/m2) | 3.1 ± 0.8 | 2.6 ± 0.5 | 0.0067 |
6 min walk test | |||
6MWD (m) | 374.0 ± 84.0 | 413.4 ± 107.1 | 0.3176 |
Lowest SPO2 (%) | 89.3 ± 3.7 | 84.5 ± 10.1 | 0.1791 |
Blood gas analysis (room air) | |||
PaO2 (mmHg) | 69.2 ± 6.8 | 72.3 ± 16.5 | 0.5462 |
PaCO2 (mmHg) | 34.3 ± 5.3 | 35.8 ± 4.4 | 0.3551 |
PvO2 (mmHg) | 36.3 ± 5.3 | 35.6 ± 3.9 | 0.6359 |
Qs/Qt | 22.8 ± 7.0 | 22.3 ± 16.1 | 0.9085 |
Pulmonary function | |||
VC%predicted | 89.1 ± 15.0 | 94.4 ± 16.6 | 0.3661 |
FEV1/FVC% | 76.9 ± 11.0 | 78.0 ± 8.7 | 0.7416 |
RV/TLC% | 37.6 ± 8.4 | 36.0 ± 6.4 | 0.5193 |
DLCO%predicted | 64.0 ± 9.3 | 61.1 ± 20.6 | 0.6741 |
DLCO/VA%predicted | 76.0 ± 13.0 | 68.2 ± 22.4 | 0.3018 |
DLCO%predicted (adjustment for Hb) | 58.2 ± 21.0 | 53.5 ± 28.5 | 0.6133 |
DLCO/VA%predicted (adjustment for Hb) | 69.0 ± 25.2 | 57.7 ± 32.0 | 0.2839 |
Blood Exam | |||
Hemoglobin (g/dl) | 13.7 ± 1.7 | 13.9 ± 2.1 | 0.8756 |
Creatinine (mg/dl) | 0.8 ± 0.3 | 0.7 ± 0.3 | 0.8844 |
Total Bilirubin (mg/dl) | 1.4 ± 0.8 | 1.0 ± 0.5 | 0.0450 |
BNP (pg/ml) | 297.0 ± 485.3 | 170.3 ± 263.2 | 0.2799 |
Smoking habits | |||
Never/ Former or current (±) | 4/6 | 14/25 | 0.8111 |
WHO FC (I/II/III/IV) | 1/6/3/0 | 4/21/10/1 | |
Comorbidity | |||
BMI ≥ 25 kg/m2 (±) | 3/8 | 9/30 | 0.7758 |
Systemic hypertension (±) | 1/10 | 3/36 | 0.8816 |
Coronary artery disease (±) | 0/11 | 2/37 | 0.3132 |
Atrial fibrillation (±) | 2/9 | 2/37 | 0.1964 |
Diabetes mellitus (±) | 1/10 | 3/36 | 0.8816 |
Number of Comorbidities | |||
Two (±) | 1/10 | 5/34 | 0.7295 |
Three (±) | 0/11 | 1/38 | 0.4783 |