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Ann Thorac Surg 1997;63:1177-1179
© 1997 The Society of Thoracic Surgeons

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Case Report

Hepatic Venous Malformation After Modified Fontan Procedure With Partial Hepatic Vein Exclusion

Children's Hospital Medical Center, Cincinnati, Ohio, and Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

Accepted for publication November 18, 1996.

	Abstract

Top Footnotes Abstract Introduction Comment References

 
Partial hepatic vein exclusion, as an alternative to baffle fenestration, has been performed as a modification of the Fontan procedure. We report a case of severe cyanosis resulting from massive right-to-left shunting via a hepatic vascular malformation that developed in a patient after a modified Fontan procedure with partial hepatic vein exclusion.

	Introduction

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The original Fontan operation [1] has undergone several modifications that have greatly improved the outcome and applicability of this operation, including the creation of a small fenestration in the atrial baffle, allowing for shunting of systemic venous blood into the pulmonary venous chamber. This results in decreased systemic venous pressure and reduced morbidity from serous effusions, and at the expense of lower arterial oxygen saturations, leads to an improvement in cardiac output and oxygen delivery [2]. This modification has led to improved survival, particularly in high-risk patients [3, 4]. In an attempt to achieve a similar physiologic advantage, an alternative modification of the Fontan operation has been to position the atrial baffle such that one or more of the hepatic veins drain to the pulmonary venous side of the atrial baffle (partial hepatic vein exclusion). We report the case of a child with hypoplastic left heart syndrome whose third-stage palliative procedure consisted of a modified Fontan operation with exclusion of the left-sided hepatic veins, in whom a large hepatic venous malformation subsequently developed, leading to excessive right-to-left shunting from the systemic venous atrium to the pulmonary venous atrium via the "excluded" hepatic veins.

The patient is a 28-month-old boy who was born with hypoplastic left heart syndrome and underwent stage 1 of the Norwood palliation procedure at 4 days of age at Children's Hospital of Philadelphia. The second stage of the Norwood procedure (superior cavopulmonary anastomosis) was performed at 7 months of age without complication. The third stage (modified Fontan operation) was performed at 18 months of age and consisted of the lateral tunnel technique. The right atrial baffle used to create the lateral tunnel was placed in such a fashion that two left-sided hepatic veins were excluded and drained to the pulmonary venous side of the baffle.

After this operation, the patient became progressively cyanotic. Initially, his postoperative oximetric saturations ranged from 80% to 85%, but dropped to 65% to 75% over the next 10 months, with associated exercise intolerance and progressive polycythemia (hemoglobin level, 20 g/dL).

A detailed echocardiogram demonstrated the cardiac anatomy consistent with his previous operations. All the hepatic veins were markedly enlarged and were connected to a large vascular malformation in the liver. The left-sided hepatic veins clearly drained to the pulmonary venous side of the atrial baffle, ie, to the left side of the baffle (Fig 1A). Color Doppler echocardiography demonstrated that blood flow in the lateral tunnel was reversed; blood from the superior vena cava flowed retrograde down the lateral tunnel toward the hepatic vascular malformation. Flow from the left-sided hepatic veins drained to the pulmonary venous side of the atrial baffle (Fig 1B). Therefore, the patient's cyanosis was due to right-to-left shunting of the systemic venous blood from the systemic venous atrium, inferior vena cava, and right-sided hepatic veins to the pulmonary venous atrium via the hepatic venous malformation.

View larger version (67K): [in this window] [in a new window]   Fig 1. . (A) Two-dimensional echocardiogram depicts position of atrial baffle (arrow), which excludes the left-sided hepatic veins. The left-sided hepatic veins are dilated and drain to the pulmonary venous side of the atrial baffle. (B) Color Doppler echocardiogram shows the direction of blood flow in the hepatic veins. Blood flow in the right hepatic vein is directed toward the liver (red), whereas blood flow in the left hepatic veins is directed away from the liver (blue), the two flows being separated by the atrial baffle (arrow).

View larger version (97K): [in this window] [in a new window]   Fig 2. . (A) Cineangiogram performed in the balloon-occluded inferior vena cava depicts a large hepatic venous malformation. Blood flow from the left-sided hepatic veins drains to the pulmonary venous side of the atrial baffle (arrow). (B) Cineangiogram performed at the junction of the superior vena cava and right pulmonary artery shows filling of both pulmonary arteries and significant retrograde flow (arrow) of contrast down the lateral tunnel toward the hepatic venous malformation.

	Comment

Top Footnotes Abstract Introduction Comment References

 
The development of vascular anomalies resulting in right-to-left shunting after Glenn and Fontan-type operations can sometimes lead to significant cyanosis [5, 6]. These shunts generally fall into two broad categories: (1) pulmonary arteriovenous malformations and (2) abnormal systemic venous channels. Pulmonary arteriovenous malformations are most likely to develop in situations where pulmonary blood flow does not include hepatic venous blood, eg, in patients with interrupted inferior venae cavae [7], suggesting a metabolic or biochemical cause. On the other hand, abnormal systemic venous channels appear to represent preexisting connections that become hemodynamically significant only after a pressure gradient is established. Our case possibly falls into the second category as exclusion of hepatic venous blood was not an issue. We speculate that there were small and insignificant venous connections, possibly embryologic remnants, within the hepatic venous system that became dilated once a pressure gradient was established between the right- and left-sided hepatic veins. After the atrial baffle was revised it equalized the pressures in all the hepatic veins, and the presence of these venous connections became hemodynamically irrelevant, and will presumably regress.

This case demonstrates an important complication of a modified Fontan procedure with partial hepatic venous exclusion. In our experience hepatic venous malformations have been seen in 8% of patients undergoing partial hepatic vein exclusion during the Fontan procedure. The incidence is significantly less if the Fontan procedure is completed by 1 year of age. Therefore, we continue to perform this modification of the Fontan procedure in selected patients (about 1 year of age) with favorable hepatic vein anatomy that allows hepatic vein exclusion without producing any obstruction of the hepatic veins.

	Footnotes

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Address reprint requests to Dr Banerjee, Division of Cardiology, Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229.

	References

Top Footnotes Abstract Introduction Comment References

 

1. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971;26:240–8.
2. Bridges ND, Castañeda AR. The fenestrated Fontan procedure. Herz 1992;17:242–5.
3. Kopf GS, Kleinman CS, Hijazi ZM, Fahey JT, Dewar ML, Hellenbrand WE. Fenestrated Fontan operation with delayed transcatheter closure of atrial septal defect. Improved results in high-risk patients. J Proc Cardiovasc Surg 1992;103:1037–47.
4. Bridges ND, Mayer JE, Lock JE, et al. Effect of baffle fenestration on outcome of the modified Fontan operation. Circulation 1992;86:1762–9.
5. Moore JW, Kirby WC, Madden WA, Gaither NS. Development of pulmonary arteriovenous malformation after modified Fontan operations. J Thorac Cardiovasc Surg 1989;95:1045–50.
6. Gatzoulis MA, Shinebourne EA, Redington AN, Rigby ML, Ho SY, Shore DF. Increasing cyanosis early after cavopulmonary connection caused by abnormal systemic venous channels. Br Heart J 1995;73:182–6.
7. 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.

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Personal Folders Download to citation manager Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Schneider, D. J. Articles by Norwood, W. I. Search for Related Content PubMed Articles by Schneider, D. J. Articles by Norwood, W. I., Jr