Case 31-2004: A four-year-old boy with hypoxemia

New England Journal of Medicine

Volumn 351, Issue 16, 2004, Pages 1667-1675

  Kinane, T Bernard ^a,b   Westra, Sjirk J ^a,b  

^a MASSACHUSETTS GENERAL HOSPITAL   (United States)

^b HARVARD MEDICAL SCHOOL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AZITHROMYCIN; CEFTRIAXONE; OXYGEN; SALBUTAMOL;

ABERNETHY MALFORMATION; ARTICLE; CASE REPORT; CLINICAL FEATURE; COMPUTER ASSISTED TOMOGRAPHY; COUGHING; DIFFERENTIAL DIAGNOSIS; ELECTROCARDIOGRAM; FEVER; HEPATOPULMONARY SYNDROME; HUMAN; HYPOXEMIA; IDIOPATHIC DISEASE; LUNG ARTERIOVENOUS SHUNT; LUNG INFILTRATE; MALE; MYCOPLASMA PNEUMONIA; OXYGEN SATURATION; PRESCHOOL CHILD; PRIORITY JOURNAL; SHUNTING; SYSTOLIC HEART MURMUR; THORAX RADIOGRAPHY; VOMITING; BLOOD; BRAIN; CLINICAL OBSERVATION; CLINICAL STUDY; CONGENITAL MALFORMATION; DIAGNOSIS; INFERIOR CAVA VEIN; KIDNEY; LIVER; NUCLEAR MAGNETIC RESONANCE IMAGING; PATHOLOGY; PATHOPHYSIOLOGY; PORTAL VEIN; RADIOGRAPHY; SCINTISCANNING; VASCULARIZATION;

ANOXEMIA; BRAIN; CHILD, PRESCHOOL; DIAGNOSIS, DIFFERENTIAL; HEPATOPULMONARY SYNDROME; HUMANS; KIDNEY; LIVER; MAGNETIC RESONANCE IMAGING; MALE; OXYGEN; PORTAL VEIN; TOMOGRAPHY, X-RAY COMPUTED; VENA CAVA, INFERIOR;

EID: 5444229131     PISSN: 00284793     EISSN: None     Source Type: Journal    
DOI: 10.1056/NEJMcpc049023     Document Type: Article

References (28)

1. West JB. Respiratory physiology - the essentials. 5th ed. New York: Williams and Wilkins, 1995:45-61.
2. Bernstein D. The cardiovascular system. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson textbook of pediatrics. 16th ed. Philadelphia: W.B. Saunders, 2000:337-409.
3. Gossage JR, Kanj G. Pulmonary arteriovenous malformations: a state of the art review. Am J Respir Crit Care Med 1998;158:643-61.
4. Lange PA, Stoller JK. The hepatopulmonary syndrome: effect of liver transplantation. Clin Chest Med 1996;17:115-23.
5. Krowka MJ, Cortese DA. Hepatopulmonary syndrome: current concepts in diagnostic and therapeutic considerations. Chest 1994;105:1528-37.
6. Churton T. Multiple aneurysms of the pulmonary artety. BMJ 1897;1:1223-5.
7. Sloan RD, Cooley RN. Congenital pulmonary arteriovenous aneurysm. Am J Roentgenol Radium Ther Nucl Med 1953;70:183-210.
8. Plauchu H, de Chadarevian JP, Bideau A, Robert JM. Age-related clinical profile of hereditary hemorrhagic telangiectasia in an epidemiologically recruited population. Am J Med Genet 1989;32:291-7.
9. Haitjema T, Disch F, Overtoom TT, Westermann CJ, Lammers JW. Screening family members of patients with hereditary hemorrhagic telangiectasia. Am J Med 1995;99:519-24.
10. Vase P, Holm M, Arendrup H. Pulmonary arteriovenous fistulas in hereditary hemorrhagic telangiectasia. Acta Med Scand 1985;218:105-9.
11. Remy J, Remy-Jardin M, Wattinne L, Deffontaines C. Pulmonary arteriovenous malformations: evaluation with CT of the chest before and after treatment. Radiology 1992;182:809-16.
12. Whyte MK, Peters AM, Hughes JM, et al. Quantification of right to left shunt at rest and during exercise in patients with pulmonary arteriovenous malformations. Thorax 1992;47:790-6.
13. Hopkins WE, Waggoner AD, Barzilai B. Frequency and significance of intrapulmonary right-to-left shunting in end-stage hepatic disease. Am J Cardiol 1992;70:516-9.
14. Rodriguez-Roisin R, Roca J, Agusti AG, Mastai R, Wagner PD, Bosch J. Gas exchange and pulmonary vascular reactivity in patients with liver cirrhosis. Am Rev Respir Dis 1987;135:1085-92.
15. Lange PA, Stoller JK. The hepatopulmonary syndrome. Ann Intern Med 1995;122:521-9.
16. Usuki N, Miyamoto T. A case of congenital absence of the intrahepatic portal vein diagnosed by MR angiography. J Comput Assist Tomogr 1998;22:728-9.
17. Badler R, Price AP, Moy L, Katz DS. Congenital portocaval shunt: CT demonstration. Pediatr Radiol 2002;32:28-30.
18. McAdams HP, Erasmus J, Crockett R, Mitchell J, Godwin JD, McDermott VG. The hepatopulmonary syndrome: radiologic findings in 10 patients. AJR Am J Roentgenol 1996;166:1379-85.
19. Lee K-N, Lee H-J, Shin WW, Webb WR. Hypoxemia and liver cirrhosis (hepatopulmonary syndrome) in eight patients: comparison of the central and peripheral pulmonary vasculature. Radiology 1999;211:549-53.
20. Engelke C, Schaefer-Prokop C, Schirg E, Freihorst J, Grubnic S, Prokop M. High-resolution CT and CT angiography of peripheral pulmonary vascular disorders. Radiographies 2002;22:739-64. [Erratum, Radiographies 2002;22:1516.]
21. Alvarez AE, Ribeiro AF, Hessel G, Baracat J, Ribeiro JD. Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 2002;34:391-4.
22. Howard ER, Davenport M. Congenital extrahepatic portocaval shunts - the Abernethy malformation. J Pediatr Surg 1997;32:494-7.
23. Kohda E, Saeki M, Nakano M, et al. Congenital absence of the portal vein in a boy. Pediatr Radiol 1999;29:235-7.
24. Srivastava D, Preminger T, Lock JE, et al. Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease. Circulation 1995;92:1217-22.
25. Lee J, Menkis AH, Rosenberg HC. Reversal of pulmonary arteriovenous malformation after diversion of anomalous hepatic drainage. Ann Thorac Surg 1998;65:848-9.
26. Ryu JK, Oh JH. Hepatopulmonary syndrome: angiography and therapeutic embolization. Clin Imaging 2003;27:97-100.
27. 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;37:192-7.
28. Taille C, Cadranel J, Bellocq A, et al. Liver transplantation for hepatopulmonary syndrome: a ten-year experience in Paris, France. Transplantation 2003;75:1482-9.