Abstract
Background and Aims
Hepatopulmonary syndrome (HPS) affects 10–32 % of patients with cirrhosis and is defined by liver abnormalities, intrapulmonary vascular dilatations (IPVDs), and abnormal oxygenation. However, published criteria for abnormal oxygenation are inconsistent. We sought to evaluate variation in oxygenation over time and to compare various diagnostic criteria for validity, based on their diagnostic stability over time and ability to identify patients with clinically relevant findings.
Methods
We retrospectively analyzed oxygenation and diffusion capacity in patients with liver abnormalities and IPVDs who had ≥2 arterial blood gases (ABGs) at the University of Toronto or Universite de Montreal. We compared the performance of nine possible oxygenation criteria for HPS and for each explored whether validity improved when requiring two consecutive abnormal ABGs on different days.
Results
Mean PaO2 was 68.4 mmHg and annual within-patient coefficient of variation 6.3 % (58 patients). Applying published criteria, 8.6–15.5 % of patients initially diagnosed with HPS no longer met the criterion for HPS on a subsequent ABG (re-classified). Requiring two consecutive abnormal ABGs on different days: (1) reduced the proportion of re-classified patients (9/9 criteria); (2) identified patients with more rapid progression in hypoxemia and greater difference in rate of progression between HPS and non-HPS (7/9 criteria); and (3) identified patients with lower diffusion and a larger difference in diffusion between HPS and non-HPS (8/9 criteria).
Conclusions
Oxygenation is variable in this population, and requiring two abnormal results might reduce misdiagnosis and better differentiate patients with and without HPS according to clinically relevant markers of disease.
Similar content being viewed by others
Abbreviations
- AaDO2 :
-
Alveolar-arterial oxygen gradient
- ABG:
-
Arterial blood gas
- CE:
-
Contrast echocardiography
- DLCO:
-
Diffusion capacity
- HPS:
-
Hepatopulmonary syndrome
- IPVDs:
-
Intrapulmonary vascular dilatations
- PaO2 :
-
Partial pressure of arterial oxygen
References
Schenk P, Schoniger-Hekele M, Fuhrmann V, Madl C, Silberhumer G, Muller C. Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterology. 2003;125:1042–1052.
Kochar R, Tanikella R, Fallon MB. Serial pulse oximetry in hepatopulmonary syndrome. Dig Dis Sci. 2011;56:1862–1868.
Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB, Committee ERSTFP-HVDS. Pulmonary-hepatic vascular disorders (PHD). [Review] [281 refs]. Eur Respir J. 2004;24:861–880.
Schenk P, Fuhrmann V, Madl C, et al. Hepatopulmonary syndrome: prevalence and predictive value of various cut offs for arterial oxygenation and their clinical consequences. Gut. 2002;51:853–859.
Hopkins WE, Waggoner AD, Barzilai B. Frequency and significance of intrapulmonary right-to-left shunting in end-stage hepatic disease. Am J Cardiol. 1992;70:516–519.
Gupta S, Bayoumi AM, Faughnan ME. Rare lung disease research: strategies for improving identification and recruitment of research participants. Chest. 2011;140:1123–1129.
Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. 2005;26:948–968.
Fallon MB, Abrams GA. Pulmonary dysfunction in chronic liver disease. Hepatology. 2000;32:859–865.
Krowka MJ, Dickson ER, Cortese DA. Hepatopulmonary syndrome. Clinical observations and lack of therapeutic response to somatostatin analogue. Chest. 1993;104:515–521.
Swanson KL, Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: impact of liver transplantation. Hepatology. 2005;41:1122–1129.
Gupta S, Castel H, Rao RV, et al. Improved survival after liver transplantation in patients with hepatopulmonary syndrome. Am J Transpl. 2010;10:354–363.
Thorson SH, Marini JJ, Pierson DJ, Hudson LD. Variability of arterial blood gas values in stable patients in the ICU. Chest. 1983;84:14–18.
Sasse SA, Chen PA, Mahutte CK. Variability of arterial blood gas values over time in stable medical ICU patients. Chest. 1994;106:187–193.
Organ Procurement and Transplantation Network. Organ Distribution: Allocation of Livers. 2011.
Arguedas MR, Abrams GA, Krowka MJ, Fallon MB. Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation. Hepatology. 2003;37:192–197.
Goldberg DS, Krok K, Batra S, Trotter JF, Kawut SM, Fallon MB. Impact of the hepatopulmonary syndrome MELD exception policy on outcomes of patients after liver transplantation: an analysis of the UNOS database Gastroenterology. 2014;146:1256–1265 e1251.
Fallon MB, Mulligan DC, Gish RG, Krowka MJ. Model for end-stage liver disease (MELD) exception for hepatopulmonary syndrome. [Erratum appears in Liver Transpl. 2008 Sep; 14(9):1386]. Liver Transpl. 2006;12:S105–S107.
Mandell MS. Hepatopulmonary syndrome and portopulmonary hypertension in the model for end-stage liver disease (MELD) era. Liver Transpl Off publication of the American Association for the Study of Liver Diseases and the Int Liver Transpl Soc. 2004;10:S54–S58.
Fallon MB, Krowka MJ, Brown RS, et al. Impact of hepatopulmonary syndrome on quality of life and survival in liver transplant candidates. Gastroenterology. 2008;135:1168–1175.
Krowka MJ. Caveats concerning hepatopulmonary syndrome.[comment]. [Review] [19 refs]. J Hepatol. 2001;34:756–758.
Fallon MB, Abrams GA. Pulmonary dysfunction in chronic liver disease [Review] [67 refs]. Hepatology. 2000;32:859–865.
Roberts KE, Kawut SM, Krowka MJ et al. Genetic risk factors for hepatopulmonary syndrome in patients with advanced liver disease Gastroenterology. 2010;139:130–139.e124.
Rodriguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome–a liver-induced lung vascular disorder [Review] [50 refs]. N Engl J Med. 2008;358:2378–2387.
Abrams GA, Sanders MK, Fallon MB. Utility of pulse oximetry in the detection of arterial hypoxemia in liver transplant candidates. Liver Transpl. 2002;8:391–396.
Martinez-Palli G, Drake BB, Garcia-Pagan JC, et al. Effect of transjugular intrahepatic portosystemic shunt on pulmonary gas exchange in patients with portal hypertension and hepatopulmonary syndrome. World J Gastroenterol. 2005;11:6858–6862.
Palma DT, Philips GM, Arguedas MR, Harding SM, Fallon MB. Oxygen desaturation during sleep in hepatopulmonary syndrome. Hepatology. 2008;47:1257–1263.
Aller R, Moya JL, Moreira V, et al. Diagnosis of hepatopulmonary syndrome with contrast transesophageal echocardiography: advantages over contrast transthoracic echocardiography. Dig Dis Sci. 1999;44:1243–1248.
Iyer VN, Swanson KL, Cartin-Ceba R, et al. Hepatopulmonary syndrome: favorable outcomes in the MELD exception era. Hepatology. 2013;57:2427–2435.
Moreira Silva H, Reis G, Guedes M, et al. A case of hepatopulmonary syndrome solved by mycophenolate mofetil (an inhibitor of angiogenesis and nitric oxide production). J Hepatol. 2013;58:630–633.
Saigal S, Choudhary N, Saraf N, et al. Excellent outcome of living donor liver transplantation in patients with hepatopulmonary syndrome: a single centre experience. Clin Transpl. 2013;27:530–534.
Enache I, Oswald-Mammosser M, Woehl-Jaegle ML, et al. Cirrhotic cardiomyopathy and hepatopulmonary syndrome: prevalence and prognosis in a series of patients. Respir Med. 2013;107:1030–1036.
Acknowledgments
We would like to thank Dr. Marie E. Faughnan for her helpful comments on this manuscript. Mr. Dhruv Nayyar has been supported by the The Keenan Research Centre in the Li Ka Shing Knowledge Institute Summer Student Program. We would also like to acknowledge Dr. Pomier-Layrargues’ mentorship and leadership. He is missed dearly.
Conflict of interest
None.
Author information
Authors and Affiliations
Corresponding author
Additional information
Gilles Pomier-Layrargues: Deceased.
Rights and permissions
About this article
Cite this article
Gupta, S., Nayyar, D. & Pomier-Layrargues, G. Variability of Oxygenation in Possible Hepatopulmonary Syndrome: Effects of Requiring Two Abnormal Arterial Blood Gas Results for Diagnosis. Dig Dis Sci 60, 1848–1855 (2015). https://doi.org/10.1007/s10620-014-3506-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10620-014-3506-7