Pulmonary Arteriovenous Malformations in Heterotaxy Syndrome: The Case for Early, Direct Hepatic Vein–to–Azygos Vein Connection

World Journal for Pediatric and Congenital Heart Surgery

Volumn 2, Issue 1, 2011, Pages 119-128

  Burstein, Danielle S ^a   Mavroudis, Constantine ^a   Puchalski, Michael D ^b   Stewart, Robert D ^a   Blanco, Carlos J ^a   Jacobs, Marshall L ^a  

^a LERNER RESEARCH INSTITUTE   (United States)

^b PRIMARY CHILDREN'S MEDICAL CENTER   (United States)

Author keywords

CHD univentricular heart; Fontan; heterotaxy; pulmonary arteriovenous malformations

Indexed keywords

EID: 84990339303     PISSN: 21501351     EISSN: 21500136     Source Type: Journal    
DOI: 10.1177/2150135110387310     Document Type: Article

References (35)

1. Jacobs ML. Complications associated with heterotaxy syndrome in Fontan patients. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2002; 5: 23–35.
2. Tchervenkov CI Jacobs ML Duca DD. Surgery for the functionally univentricular heart in patients with visceral heterotaxy. Cardiol Young. 2006; 16(Supplement 1):72–79.
3. Rodriguez-Roisin R Krowka MJ. Hepatopulmonary syndrome: a liver-induced lung vascular disorder. N Engl J Med. 2008; 358: 2378–2387.
4. Grifka RG Preminger TJ. Vascular anomalies. In: Allen HD, Gutgesell HP, Clark EB, Driscoll DJ,eds. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents, Including the Fetus and Young Adult. Vol 1, 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2001: 698–699.
5. Amodeo A Di Carlo D Grigioni M De Santis M Di Donato RM, Early primary Kawashima operation combined with direct hepatic vein-to-azygos vein connection: a new logical approach. J Thorac Cardiovasc Surg. 2005; 129: 949–950.
6. Baskett RJ Ross DB Warren AE Sharratt GP Murphy DA. Hepatic vein to the azygous vein anastomosis for pulmonary arteriovenous fistulae. Ann Thorac Surg. 1999; 68: 232–233.
7. Brown JW Ruzmetov M Vijay P Rodefeld MD Turrentine MW. Pulmonary arteriovenous malformations in children after the Kawashima operation. Ann Thorac Surg. 2005; 80: 1592–1596.
8. Choi YH Lee W Cheon JE, et al. CT findings in unilateral hepatopulmonary syndrome after the Fontan operation. Pediatr Radiol. 2009; 39: 336–342.
9. Ichikawa H Fukushima N Ono M, et al. Resolution of pulmonary arteriovenous fistula by redirection of hepatic venous blood. Ann Thorac Surg. 2004; 77: 1825–1827.
10. Imoto Y Sese A Joh K. Redirection of hepatic venous flow for the treatment of pulmonary arteriovenous malformations after Fontan operation. Pediatr Cardiol. 2006; 27: 490–492.
11. Kaneko Y Murakami A Miyamoto T Takamoto S. Hepatic vein-to-azygous vein connection in a patient with interrupted inferior vena cava. Eur J Cardiothorac Surg. 2002; 21: 582–584.
12. Kim SJ Bae EJ Lee JY Lim HG Lee C Lee CH. Inclusion of hepatic venous drainage in patients with pulmonary arteriovenous fistulas. Ann Thorac Surg. 2009; 87: 548–554.
13. Knight WB Mee RB. A cure for pulmonary arteriovenous fistulas? Ann Thorac Surg. 1995; 49: 999–1001.
14. McElhinney DB Kreutzer J Lang P Mayer JE Jr del Nido PJ Lock JE. Incorporation of hepatic vein into cavopulmonary circulation in patients with heterotaxy and pulmonary arteriovenous malformations after a Kawashima procedure. Ann Thorac Surg. 2005; 80: 1597–1603.
15. Nakamura Y Yagihara T Kagisaki K Hagino I Kobayashi J. Pulmonary arteriovenous malformations after a Fontan operation in the left isomerism and absent inferior vena cava. Eur J Cardiothorac Surg. 2009; 36: 69–76.
16. Pike NA Vricella LA Feinstein JA Black MD Reitz BA. Regression of severe pulmonary arteriovenous malformations after Fontan revision and “hepatic factor” rerouting. Ann Thorac Surg. 2004; 78: 697–699.
17. Shah MJ Rychik J Fogel MA Murphy JD Jacobs ML. Pulmonary AV malformations after superior cavopulmonary connection: resolution after inclusion of hepatic veins in the pulmonary circulation. Ann Thorac Surg. 1997; 63: 960–963.
18. Steinberg J Alfieris GM Brandt B 3rd, et al. New approach to the surgical management of pulmonary arteriovenous malformations after cavopulmonary anastomosis. Ann Thorac Surg. 2003; 75: 1640–1642.
19. Uemura H Yagihara T Hattori R Kawahira Y Tsukano S Watanabe K. Redirection of hepatic venous drainage after total cavopulmonary shunt in left isomerism. Ann Thorac Surg. 1999; 68: 1731–1735.
20. Wu IH Nguyen KH. Redirection of hepatic drainage for treatment of pulmonary arteriovenous malformations following the Fontan procedure. Pediatr Cardiol. 2006; 27: 519–522.
21. Jacobs JP Anderson RH Weinberg P, et al. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young. 2007;(Supplement 2):1–28.
22. Kawashima A. Cavopulmonary shunt and pulmonary arteriovenous malformations. Ann Thorac Surg. 1997; 63: 930–932.
23. 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: 1217–1222.
24. Lee J Menkis AH Rosenberg HC. Reversal of pulmonary arteriovenous malformation after diversion of anomalous hepatic drainage. Ann Thorac Surg. 1998; 65: 848–849.
25. Cloutier A Ash JM Smallhorn JF, et al. Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistula. Circulation. 1985; 72(3):471–479.
26. Duncan BW Desai S. Pulmonary arteriovenous malformations after cavopulmonary anastomosis. Ann Thorac Surg. 2003; 76: 1759–1766.
27. Kawashima Y Kitamura S Matsuda H Shimazaki Y Nakano S Hirose H. Total cavopulmonary shunt operation in complex cardiac anomalies: a new operation. J Thorac Cardiovasc Surg. 1984; 87: 74–81.
28. Donofrio MT Jacobs ML Norwood WI Rychik J. Early changes in ventricular septal defect size and ventricular geometry in the single left ventricle after volume-unloading surgery. J Am Coll Cardiol. 1995; 26: 1008–1015.
29. Kim SJ Bae EJ Cho DJ, et al. Development of pulmonary arteriovenous fistulas after bidirectional cavopulmonary shunt. Ann Thorac Surg. 2000; 70: 1918–1922.
30. Kim SJ Kim WH Lim HG Lee CH Lee JY. Improving results of the Fontan procedure in patients with heterotaxy syndrome. Ann Thorac Surg. 2006; 82: 1245–1251.
31. Walker PG Howe TT Davies RL Fisher J Watterson KG. Distribution of hepatic venous blood in the total cavo-pulmonary connection: an in vitro study. Eur J Cardiothorac Surg. 2000; 17: 658–665.
32. Bartz PJ Driscoll DJ Dearani JA, et al. Early and later results of the modified Fontan operation for heterotaxy syndrome: 30 years of experience in 142 patients. J Am Coll Cardiol. 2006; 48: 2301–2305.
33. Koeppen BM Stanton BA. Hepatic circulation. Keoppen & Stanton: Berne and Levy Physiology. 6th ed. Philadelphia, PA: Mosby Elsevier; 2010: 340–341.
34. Konstantinov IE Puga FJ Alexi-Meskishvili VV. Thrombosis of intracardiac and extracardiac conduits after modified Fontan operation in patients with azygous continuation of the inferior vena cava. Ann Thorac Surg. 2001; 72: 1641–1644.
35. Lopez FE van den Heuvel F Pieper PG Waterbolk YW Ebels T. Off-pump connection of the hepatic to the azygos vein through a lateral thoracotomy for relief of arterio-venous fistulas after a Kawashima procedure. Cardiol Young. 2008; 18: 311–315.