Table 1.
Characteristics | HPS | POPH |
---|---|---|
Clinical issue* | Arterial hypoxemia caused by IPVD | Pulmonary artery hypertension |
Diagnostic criteria† | PaO2 < 80 mm Hg | mPAP > 25 mm Hg; PVR > 3 wood units |
LD | Usually cirrhosis | Always portal hypertension |
Severity | Poor correlation with LD | Poor correlation with LD |
Frequency seen | 5%‐32% | 5%‐10% |
Medical treatments‡ | Wear supplemental oxygen | PV medications |
5‐Year survival rate | 23% (no treatments) | 4%‐14%; 40% survival rate with PV therapy only |
LT§ | An “indication” | A “contraindication” if mPAP > 45 mm Hg |
Treatment outcomes‖ | Complete resolution with LT | Unpredictable, 50% resolution with LT |
IPVDs were determined by contrast‐enhanced transthoracic echocardiography.
PaO2 determined by arterial blood gas in the sitting position breathing room air; right heart catheterization measures/calculates mPAP and PVR, respectively.
Several available for POPH, but only one randomized, placebo‐controlled trial conducted; no controlled trials in HPS to date.
Increased LT risk. Expedited LT consideration if pretransplant PaO2 < 60 mm Hg (HPS); if mPAP < 35 mm Hg with PV therapy (POPH).
Time resolution of HPS related to severity of hypoxemia; PV therapy in POPH can be discontinued in 50% after LT.