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Table 3 Baseline characteristics of patients from the study at Chiba University Hospital (PoPH and I/H-PAH)

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

  1. Data provided as mean ± SD or n
  2. HCV, hepatitis c virus; mPAP, mean pulmonary arterial pressure; PVR, pulmonary vascular resistance; PAWP, pulmonary arterial wedge pressure; RAP, right atrial pressure; CI, cardiac index; PvO2, mixed venous oxygen pressure; 6MWD, 6-min walk distance; VC, vital capacity; FEV1, forced expiratory volume in 1 s; RV, residual volume; TLC; total lung capacity; DLco; diffusing capacity of the lung for carbon monoxide; BNP, brain natriuretic peptide; WHO FC, World Health Organization functional class