Table 2.
HPS: A Chronology of Selected Milestones
| Year | First Author | Observation/Contribution |
|---|---|---|
| 1884 | Fluckiger1 | Cyanosis, clubbing in cirrhosis first described |
| 1938 | Keys30 | Hypoxemia in cirrhosis caused by rightward shift of the hemoglobin/oxygen (Hgb‐O2) curve |
| 1953 | Wilson31 | Hypoxemia in cirrhosis due to “venous admixture” rather than Hgb‐O2 curve shift |
| 1956 | Rydel32 | First clinic pathological case report of HPS |
| 1957 | Calabresi33 | Unusual portal to pulmonary vein connections described |
| 1960 | Rodman34 | “Unsaturation of arterial blood” due to venous admixture common in cirrhosis |
| 1963 | Mellemgaard35 | Portal to pulmonary vein connections unlikely to contribute to hypoxemia |
| 1966 | Berthelot36 | Lung pathologies of vascular dilatation and possible angiogenesis described |
| 1968 | Starzl13 | First LTs attempted in the setting of presumed intrapulmonary shunting |
| 1977 | Kennedy2 | The term “hepatopulmonary syndrome” first coined |
| 1984 | Van Thiel14 | HPS with PaO2 < 50 mm Hg an “absolute contraindication” to LT |
| 1987 | Krowka37 | Small series using almitrine bismesylate for HPS; no improvement in PaO2 |
| 1988 | Maddrey38 | HPS with PaO2 < 50 mm Hg a “relative contraindication” to LT |
| 1989 | Edell39 | MIGET characterized reasons for the hypoxemia of HPS |
| 1990 | Krowka40 | Screening for HPS using contrast‐enhanced transthoracic echocardiography described |
| 1990 | Stoller41 | HPS resolves in an adult with PBC after LT |
| 1992 | LaBerge42 | HPS resolves in two children after LT |
| 1992 | Chang4 | Common bile duct ligation experimental model in a rat developed for HPS |
| 1996 | Krowka15 | Mayo cases and literature review: progressive HPS an “indication” for HPS |
| 1997 | Fallon5 | First describes nitric oxide implication in the rat model of HPS |
| 1998 | Whyte43 | Use of 99mTcMAA lung‐brain scanning quantifies HPS severity |
| 1999 | Martinez44 | Association between HPS and other common respiratory disorders described |
| 1999 | Egawa45 | Living donor LT for HPS reported and excellent long‐term outcomes |
| 2000 | Schenk46 | Small series showing IV methylene blue improved HPS hypoxemia in intensive care unit setting |
| 2003 | Taille47 | Resolution of HPS post‐LT dependent on severity pretransplant |
| 2003 | Schenk48 | HPS reported as an independent risk for poor outcome in liver disease |
| 2004 | Rodriguez‐Roisin49 | ERS consensus committee defines HPS diagnostic criteria |
| 2005 | Swanson50 | 5‐Year natural history (without transplant) of HPS described |
| 2006 | Fallon16 | MELD exception criteria (PaO2 < 60 mm Hg) for HPS began |
| 2006 | Gomez51 | Nebulized l‐NAME decreased exhaled nitric oxide but does not improve PaO2 in HPS |
| 2008 | Rodriguez‐Roisin52 | New England Journal of Medicine review article: hepatopulmonary syndrome: liver‐induced lung vascular disorder |
| 2008 | Fleming53 | Use of ECMO after LT for refractory HPS |
| 2010 | Roberts54 | Genetic risk factors for HPS described |
| 2010 | Gupta17 | Excellent LT outcome in severe HPS (PaO2 < 50 mm Hg) |
| 2013 | Iyer18 | Excellent LT outcomes for HPS in the era of MELD exception |
| 2014 | Goldberg55 | Impact of MELD exception for HPS described from the UNOS database |
| 2016 | Krowka26 | ILTS consensus practice guidelines for HPS published |
| 2019 | Raevens56 | Excellent HPS outcomes after LT: Eurotransplant experience |
| 2019 | Kawut11 | First randomized, controlled trial in HPS (sorafenib); no improvement in PaO2 |