Table 1 Previous literature reporting exercise testing in patients with HPS

Thorens et al., 1992 [11]

HPS case report (n = 1); constant work rate test*

• Worsening physiologic shunt (from 12% at rest to 26% with exercise)

Epstein et al., 1998 [7]

HPS (n = 5) vs cirrhosis^† (n = 19); incremental cycle ergometry

• Reduced VO_2peak (55% predicted in HPS vs 72% predicted in cirrhosis)

• Progressive exercise hypoxemia

• Earlier onset of the anaerobic threshold

• Elevated dead space ventilation

Whyte et al., 1998 [9]

HPS (n = 8); incremental cycle ergometry

• Progressive exercise desaturation

• Diminished achieved workload (mean 48% predicted)

• Reduced mixed venous oxygen content

Nusair et al., 2005 [8]

HPS case report (n = 1); incremental cycle ergometry

• Reduced VO_2peak (41% predicted)

• Progressive exercise hypoxemia

• Marked dyspnea

• Worsening physiologic shunt

Faustini-Pereira et al., 2015 [12]

HPS (n = 92) vs cirrhosis^‡ (n = 86); modified Bruce protocol*

• Reduced VO_2peak (80.2% predicted in HPS vs 86.7% predicted in cirrhosis)

• Reduced 6-min walk distance (341 m in HPS vs 416 m in cirrhosis)