We report a surgical approach using hepatic vein–to–azygos vein connection without cardiopulmonary bypass or deep hypothermic circulatory arrest in a patient with heterotaxy syndrome and interrupted inferior vena cava with persistence of pulmonary arteriovenous malformations (PAVMs) after previous Fontan completion. We advocate early performance of hepatic vein–to–azygos vein connection following the Kawashima operation for heterotaxy with functionally univentricular heart and interrupted inferior vena cava. We review the physiology of heterotaxy syndrome with congenital heart disease and justify our approach in the context of a review of previous surgical strategies used in this patient population.
JacobsML. Complications associated with heterotaxy syndrome in Fontan patients. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2002;5:23–35.
2.
TchervenkovCIJacobsMLDucaDD. Surgery for the functionally univentricular heart in patients with visceral heterotaxy. Cardiol Young. 2006;16(Suppl 1):72–79.
3.
Rodriguez-RoisinRKrowkaMJ. Hepatopulmonary syndrome: a liver-induced lung vascular disorder. N Engl J Med. 2008;358:2378–2387.
4.
GrifkaRGPremingerTJ.Vascular anomalies. In:
AllenHDGutgesellHPClarkEBDriscollDJ 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.
AmodeoADi CarloDGrigioniMDe SantisMDi DonatoRM. 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.
BaskettRJRossDBWarrenAESharrattGPMurphyDA. Hepatic vein to the azygous vein anastomosis for pulmonary arteriovenous fistulae. Ann Thorac Surg. 1999;68:232–233.
7.
BrownJWRuzmetovMVijayPRodefeldMDTurrentineMW. Pulmonary arteriovenous malformations in children after the Kawashima operation. Ann Thorac Surg. 2005;80:1592–1596.
8.
ChoiYHLeeWCheonJE. CT findings in unilateral hepatopulmonary syndrome after the Fontan operation. Pediatr Radiol. 2009;39:336–342.
9.
IchikawaHFukushimaNOnoM. Resolution of pulmonary arteriovenous fistula by redirection of hepatic venous blood. Ann Thorac Surg. 2004;77:1825–1827.
10.
ImotoYSeseAJohK. Redirection of hepatic venous flow for the treatment of pulmonary arteriovenous malformations after Fontan operation. Pediatr Cardiol. 2006;27:490–492.
11.
KanekoYMurakamiAMiyamotoTTakamotoS. Hepatic vein-to-azygous vein connection in a patient with interrupted inferior vena cava. Eur J Cardiothorac Surg. 2002;21:582–584.
12.
KimSJBaeEJLeeJYLimHGLeeCLeeCH. Inclusion of hepatic venous drainage in patients with pulmonary arteriovenous fistulas. Ann Thorac Surg. 2009;87:548–554.
13.
KnightWBMeeRB. A cure for pulmonary arteriovenous fistulas?Ann Thorac Surg. 1995;49:999–1001.
14.
McElhinneyDBKreutzerJLangPMayerJEJrdel NidoPJLockJE. 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.
NakamuraYYagiharaTKagisakiKHaginoIKobayashiJ. 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.
PikeNAVricellaLAFeinsteinJABlackMDReitzBA. Regression of severe pulmonary arteriovenous malformations after Fontan revision and “hepatic factor” rerouting. Ann Thorac Surg. 2004;78:697–699.
17.
ShahMJRychikJFogelMAMurphyJDJacobsML. 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.
SteinbergJAlfierisGMBrandtB 3rd. New approach to the surgical management of pulmonary arteriovenous malformations after cavopulmonary anastomosis. Ann Thorac Surg. 2003;75:1640–1642.
19.
UemuraHYagiharaTHattoriRKawahiraYTsukanoSWatanabeK. Redirection of hepatic venous drainage after total cavopulmonary shunt in left isomerism. Ann Thorac Surg. 1999;68:1731–1735.
20.
WuIHNguyenKH. Redirection of hepatic drainage for treatment of pulmonary arteriovenous malformations following the Fontan procedure. Pediatr Cardiol. 2006;27:519–522.
21.
JacobsJPAndersonRHWeinbergP. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young. 2007;(Suppl 2):1–28.
22.
KawashimaA. Cavopulmonary shunt and pulmonary arteriovenous malformations. Ann Thorac Surg. 1997;63:930–932.
23.
SrivastavaDPremingerTLockJE. Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease. Circulation. 1995;92:1217–1222.
24.
LeeJMenkisAHRosenbergHC. Reversal of pulmonary arteriovenous malformation after diversion of anomalous hepatic drainage. Ann Thorac Surg. 1998;65:848–849.
25.
CloutierAAshJMSmallhornJF. 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.
DuncanBWDesaiS. Pulmonary arteriovenous malformations after cavopulmonary anastomosis. Ann Thorac Surg. 2003;76:1759–1766.
27.
KawashimaYKitamuraSMatsudaHShimazakiYNakanoSHiroseH. Total cavopulmonary shunt operation in complex cardiac anomalies: a new operation. J Thorac Cardiovasc Surg. 1984;87:74–81.
28.
DonofrioMTJacobsMLNorwoodWIRychikJ. 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.
KimSJBaeEJChoDJ. Development of pulmonary arteriovenous fistulas after bidirectional cavopulmonary shunt. Ann Thorac Surg. 2000;70:1918–1922.
30.
KimSJKimWHLimHGLeeCHLeeJY. Improving results of the Fontan procedure in patients with heterotaxy syndrome. Ann Thorac Surg. 2006;82:1245–1251.
31.
WalkerPGHoweTTDaviesRLFisherJWattersonKG. Distribution of hepatic venous blood in the total cavo-pulmonary connection: an in vitro study. Eur J Cardiothorac Surg. 2000;17:658–665.
32.
BartzPJDriscollDJDearaniJA. 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.
KoeppenBMStantonBA.Hepatic circulation.
Keoppen & Stanton: Berne and Levy Physiology. 6th ed.Philadelphia, PA: Mosby Elsevier; 2010:340–341.
34.
KonstantinovIEPugaFJAlexi-MeskishviliVV. 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.
LopezFEvan den HeuvelFPieperPGWaterbolkYWEbelsT. 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.
