Table 5 Baseline characteristics, treatment at the final visit, and outcome of patients with PoPH
From: The clinical characteristics, treatment, and survival of portopulmonary hypertension in Japan
Case | Etiology | Sex | Child–Pugh | mPAP (mmHg) | PVR (dyne/s/cm−5) | CI (L/min/m2) | 6MWD (m) | Medical therapy (mg/day) | Discontinuation by side effects | Follow-up period (years) | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|
1 | Cryptogenic | M | – | 40 | 621.6 | 2.99 | None | 2.4 | Died (sepsis) | ||
2 | Biliary atresia | F | A | 56 | 563.9 | 3.57 | 525 | None | 15.6 | ||
3 | Extra-hepatic portal obstruction | F | – | 47 | 577.2 | 3.19 | Macitentan(10), Sildenafil(60), Selexipag (2.4) | Tadarafil (low blood pressure, headache), Riociguat (low blood pressure) | 17.2 | ↓mPAP, PVR | |
4 | HCV | F | C | 46 | 537.5 | 3.59 | 370 | None | 0.3 | ||
5 | Extrahepatic portal obstruction | F | – | 53 | 614.0 | 3.24 | 360 | Ambrisentan (10) | Sildenafil (headache, dizziness), Riociguat (hepatic dysfunction), Selexipag (hepatic dysfunction, nausea, tinnitus, malaise, face redness) | 11.3 | ↓mPAP, PVR |
6 | HCV | F | A | 33 | 339.3 | 4.01 | 306 | None | 2 | ||
7 | HCV | F | A | 44 | 477.0 | 3.86 | 436 | Macitentan (5), Sildenafil (60) | Ambrisentan (thrombocytopenia) | 10.9 | |
8 | PBC and Autoimmune | F | A | 71 | 2501.4 | 1.48 | 267 | Sildenafil (60) | 0.2 | Died (heart failure) | |
9 | HCV | M | A | 60 | 1213.8 | 1.94 | 430 | Riociguat (6) | Tadarafil (hepatic dysfunction) | 14.7 | ↓mPAP, PVR |
10 | Cryptogenic | F | – | 45 | 678.3 | 2.67 | 275 | Bosentan (125), Sildenafil (60) | 5.6 | ↑mPAP, PVR Died (heart failure) | |
11 | Extrahepatic portal obstruction | M | – | 43 | 373.5 | 4.00 | 397 | Macitentan (10) | 3.7 | ↓mPAP, PVR |