Abstract
Background
Hepatopulmonary syndrome (HPS) is defined as an arterial oxygenation defect induced by intrapulmonary vascular dilatation (IPVD) associated with hepatic disease. The prevalence and clinical characteristics of HPS in portal hypertensive children is not well characterized.
Aims
The aim of this study was to investigate the prevalence and clinical characteristics of HPS in 40 portal hypertensive children.
Methods
We studied 40 children (11 girls and 29 boys; mean age, 111 months ± 52 months; range, 24–216 months) with portal hypertension (24 cirrhotic, 16 non-cirrhotic) for the presence of HPS using blood gas analysis, contrast-enhanced echocardiography (CEE), and Tc99m-macroaggregated albumin scintigraphy. Clinical and laboratory characteristics of patients were recorded. HPS was considered to be present in a patient with hypoxemia and/or an elevated alveolar–arterial oxygen gradient (PAaO2) ≥15 mmHg) and positive CEE and/or scintigraphy.
Results
Elevated PAaO2 was detected in 7 of 24 patients with cirrhosis. Four of them also had IPVD with CEE. An intrapulmonary shunt in Tc99m-MAA with CEE was shown in one patient. A diagnosis of HPS was made in 16.7% of the patients with cirrhosis. Cirrhotic patients without IPVD had significantly better hepatic function and lower pediatric end-stage liver disease scores. Although, 2 of the 16 patients with non-cirrhotic portal hypertension had elevated PAaO2, none of them showed IPVD. One normoxemic patient in the non-cirrhotic portal hypertension group showed IPVD with CEE. None of the non-cirrhotic patients fulfilled the diagnostic criteria of HPS.
Conclusion
Hepatopulmonary syndrome particularly occurs in cirrhotic portal hypertensive patients with severe hepatic dysfunction.
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References
Krowka MJ, Cortese DA. Hepatopulmonary syndrome: an evolving perspective in the era of liver transplantation. Hepatology. 1990;11:138–142.
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.
Barbe T, Losay J, Grimon G, et al. Pulmonary arteriovenous shunting in children with liver disease. J Pediatr. 1995;126:571–579.
Sasaki T, Hasegawa T, Kimura T, Okada A, Mushiake S, Matsushita T. Development of intrapulmonary arteriovenous shunting in postoperative biliary atresia: evaluation by contrast-enhanced echocardiography. J Pediatr Surg. 2000;35:1647–1650. (Epub ahead of print). doi:10.1053/jpsu.2000.18343
Tumgor G, Arikan C, Yuksekkaya HA, et al. Childhood cirrhosis, hepatopulmonary syndrome and liver transplantation. Pediatr Transplant. 2008;12:353–357. (Epub ahead of print). doi:10.1111/j.1399-3046.2007.00807.x
Whitworth JR, Ivy DD, Gralla J, Narkewicz MR, Sokol RJ. Pulmonary vascular complications in asymptomatic children with portal hypertension. J Pediatr Gastroenterol Nutr. 2009;49:607–612. (Epub ahead of print). doi:10.1097/MPG.0b013e3181a5267d
de Franchis R. Evolving consensus in portal hypertension. Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. J Hepatol. 2005;43:167–176. (Epub ahead of print). doi:10.1016/j.jhep.2005.05.009
Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB. Pulmonary-hepatic vascular disorders (PHD). Eur Respir J. 2004;24:861–880. (Epub ahead of print). doi:10.1183/09031936.04.00010904
Krowka MJ, Tajik AJ, Dickson ER, Wiesner RH, Cortese DA. Intrapulmonary vascular dilatations (IPVD) in liver transplant candidates. Screening by two-dimensional contrast-enhanced echocardiography. Chest. 1990;97:1165–1170.
Lenci I, Alvior A, Manzia TM, Toti L, Neuberger J, Steeds R. Saline contrast echocardiography in patients with hepatopulmonary syndrome awaiting liver transplantation. J Am Soc Echocardiogr. 2009;22:89–94. (Epub ahead of print). doi:10.1016/j.echo.2008.09.020
Grimon G, Andre L, Bernard O, Raffestin B, Desgrez A. Early radionuclide detection of intrapulmonary shunts in children with liver disease. J Nucl Med. 1994;35:1328–1332.
McDiarmid SV. Treatment of end-stage liver disease. In: Walker WA, Goulet O, Kleinman RE, Sherman PM, Shneider BL, Sanderson IR, eds. Pediatric Gastrointestinal Disease. 4th ed. ON: B. C. Decker; 2004:1508–1531.
Bernard O. Pulmonary arteriovenous shunting and pulmonary artery hypertension in children with liver disease. Pediatr Pulmonol Suppl. 1999;18:88–90.
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.
Gupta D, Vijaya DR, Gupta R, et al. Prevalence of hepatopulmonary syndrome in cirrhosis and extrahepatic portal venous obstruction. Am J Gastroenterol. 2001;96:3395–3399. (Epub ahead of print). doi:10.1111/j.572-0241.2001.05274.x
De BK, Sen S, Biswas PK, et al. Occurrence of hepatopulmonary syndrome in Budd-Chiari syndrome and the role of venous decompression. Gastroenterology. 2002;122:897–903.
Mahmoodi E, Kianifar HR, Partovi S, Mafinejad S, Bozorgnia A. Intrapulmonary arteriovenous shunt in children with chronic liver disease: clinical features, laboratory data and outcome. Indian J Gastroenterol. 2008;27:16–18.
Noli K, Solomon M, Golding F, Charron M, Ling SC. Prevalence of hepatopulmonary syndrome in children. Pediatrics. 2008;121:e522–e527. (Epub ahead of print). doi:10.1542/peds.2007-1075
Kim J, Kim K, Ko J, Lee Y, Lee S. Intrapulmonary shunt in the course of pediatric liver transplantation. Transpl Proc. 2008;40:2512–2514. (Epub ahead of print). doi:10.1016/j.transproceed.2008.07.013
Herve P, Le Pavec J, Sztrymf B, Decante B, Savale L, Sitbon O. Pulmonary vascular abnormalities in cirrhosis. Best Pract Res Clin Gastroenterol. 2007;21:141–159. (Epub ahead of print). doi:10.1016/j.bpg.2006.07.011
Mandell MS. Clinical controversies surrounding the diagnosis and treatment of hepatopulmonary syndrome. Minerva Anestesiol. 2007;73:347–355.
Rodriguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome: a liver-induced lung vascular disorder. N Engl J Med. 2008;358:2378–2387. (Epub ahead of print). doi:10.1056/NEJMra0707185
Krowka MJ. Hepatopulmonary syndrome: a prospective study of relationships between severity of liver disease, PaO2 response to 100% oxygen, and brain uptake after 99mTc MAA lung scanning. Chest. 2000;118:615–624. (Epub ahead of print). doi:10.1378/chest.118.3.615
Rodriguez-Roisin R, Agusti AG, Roca J. The hepatopulmonary syndrome: new name, old complexities. Thorax. 1992;47:897–902.
Abrams GA, Nanda NC, Dubovsky EV, Krowka MJ, Fallon MB. Use of macroaggregated albumin lung perfusion scan to diagnose hepatopulmonary syndrome: a new approach. Gastroenterology. 1998;114:305–310.
Fischer CH, Campos O, Fernandes WB, et al. Role of contrast-enhanced transesophageal echocardiography for detection of and scoring intrapulmonary vascular dilatation. Echocardiography. 2010;27:1233–1237. (Epub ahead of print). doi:10.1111/j.1540-8175.2010.01228.xECHO1228
Ho V. Current concepts in the management of hepatopulmonary syndrome. Vasc Health Risk Manag. 2008;4:1035–1041.
Swanson KL, Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: impact of liver transplantation. Hepatology. 2005;41:1122–1129. (Epub ahead of print). doi:10.1002/hep.20658
Lenci I, Alvior A, Manzia T, Toti L, Neuberger J, Steeds R. Saline contrast echocardiography in patients with hepatopulmonary syndrome awaiting liver transplantation. J Am Soc Echocardiogr. 2009;22:89–94. (Epub ahead of print). doi:10.1016/j.echo.2008.09.020
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Sari, S., Oguz, D., Sucak, T. et al. Hepatopulmonary Syndrome in Children with Cirrhotic and Non-Cirrhotic Portal Hypertension: A Single-Center Experience. Dig Dis Sci 57, 175–181 (2012). https://doi.org/10.1007/s10620-011-1832-6
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DOI: https://doi.org/10.1007/s10620-011-1832-6