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J Thorac Cardiovasc Surg 1995;109:180-181
© 1995 Mosby, Inc.

BRIEF COMMUNICATIONS

Intrahepatic steal after Fontan operation with partial hepatic exclusion

Portland, Ore.

From the Children's Cardiac Center of Oregon, P.C., 9155 S.W. Barnes Road, Suite 240, Portland, OR 97225-6689.

We would like to share our experience with a patient in whom a progressive right-to-left shunt developed because of intrahepatic steal

A 3 -year-old child with hypoplastic left heart syndrome (HLHS) had undergone the Norwood stage I procedure as a neonate and a stage II (bidirectional Glenn shunt) several months later. At the age of 2 years she was treated with the complete Fontan operation (Norwood stage III), lateral tunnel, and hepatic vein exclusion by partition of the inferior vena cava (IVC). The left hepatic vein had been excluded from the tunnel and was draining to the common atrium. The right hepatic vein drained into the IVC. The child progressed well and was discharged without overt cyanosis or evidence of a right-to-left shunt.

All of her previous treatment was done at another institution, and she was brought to our center for follow-up. Over a period of 8 months increasing cyanosis and polycythemia developed, and the patient underwent cardiac catheterization. The resting arterial saturation was in the low seventies and fell further upon exertion. Cardiac catheterization revealed no baffle leaks. The right hepatic vein was in continuity with the IVC and the lateral tunnel. Contrast medium filled the pulmonary arteries from the IVC (Fig. 1). A slightly delayed view showed that contrast medium also flowed retrogradely from the IVC into the right hepatic vein and then into the hepatic tissue. It was noticed that after the contrast medium filled the hepatic sinusoidal bed, it coalesced to fill the left hepatic vein and then the common atrium, thus showing a steal phenomenon through the liver (Fig. 2). This hepatic steal created a right-to-left shunt because of the hepatic vein exclusion that had been done with the Norwood stage III operation. The relatively lower pressure in the left hepatic vein and atrium was "stealing" from the Fontan circulation, which accounted for the increasing cyanosis. Presumably, the interconnecting hepatic venous network allowed this to occur. In addition to this intrahepatic right-to-left shunt, the effective pulmonary blood flow also was reduced by this steal (Fig. 3).

View larger version (86K): [in this window] [in a new window]   Fig. 1. RPA, Right pulmonary artery; LPA, left pulmonary artery; IVC, inferior vena cava; RHV, right hepatic vein.

View larger version (83K): [in this window] [in a new window]   Fig. 2. LHV, Left hepatic vein.

View larger version (24K): [in this window] [in a new window]   Fig. 3. RV, Right ventricle.

In an attempt to reduce effusions, liver congestion, and other adverse effects of the Fontan operation, the Philadelphia group ¹ introduced partial hepatic vein exclusion by partitioning the IVC. Although this modification is attractive for its lessening of the morbidity of the Fontan operation, due consideration should be given to the possibility that late hepatic steal may occur. Our patient raises the spectre of late malfunction of the Fontan circulation after hepatic vein exclusion.

Footnotes

J THORAC CARDIOVASC SURG 1995;109:180-1

References

1. Jacobs ML, Norwood WT. Fontan operation: influence of modifications on morbidity and mortality. In: Abstracts, Thirtieth Anniversary Meeting. The Society of Thoracic Surgeons, 1993-1994:44.
   

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This Article Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Jeffrey S. Swanson Hagop Hovaguimian Anthony P. Furnary Albert Starr Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Rao, I. M. Articles by Starr, A. Search for Related Content PubMed PubMed Citation Articles by Rao, I. M. Articles by Starr, A.