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Ann Thorac Surg 1999;68:232-233
© 1999 The Society of Thoracic Surgeons

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Case Reports

Hepatic vein to the azygous vein anastomosis for pulmonary arteriovenous fistulae

^a Cardiovascular Surgery, IWK Grace Health Centre, Halifax, Nova Scotia, Canada

Address reprint requests to Dr Murphy, Cardiovascular Surgery, IWK Grace Health Centre, 5850/5980 University Ave, PO Box 3070, Halifax, NS, B3J 3G9 Canada
e-mail: dross{at}iwkgrace.ns.ca

	Abstract

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Pulmonary arteriovenous fistulae after a cavopulmonary anastomosis have been reported to resolve after hepatic venous return is included in the pulmonary circulation. We report a case in which the hepatic veins were redirected to the pulmonary circulation by connecting them directly to the azygous continuation of the inferior vena cava that had previously been connected to the right pulmonary artery. The patient’s arterial saturation of 71% increased to 92% after 6 months.

	Introduction

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Pulmonary arteriovenous fistulae are reported to occur in 20% to 32% of patients after cavopulmonary anastomosis [1–3]. This is most important in patients who have interruption of the inferior vena cava when the cavopulmonary anastomosis has been considered to be the definitive procedure [2, 4]. A few cases have been reported in which the hepatic blood flow has been subsequently diverted to the pulmonary arteries [4–7]. In these patients, the arterial saturations are reported to have improved over subsequent months. In these cases, intracardiac or extracardiac conduits or baffles were used to redirect hepatic venous return to the pulmonary circulation. We report a patient in whom redirection of the hepatic venous return to the pulmonary circulation was achieved by anastomosis of the hepatic veins to the azygous continuation of the inferior vena cava.

A 2-month-old cyanosed infant was diagnosed with visceral situs ambiguus with levocardia. There was left isomerism of the atrial appendages with an ambiguus atrioventricular connection via a common atrioventricular valve to a ventricular chamber of right ventricular type and right hand topology; the left ventricle was rudimentary. The ventriculo-arterial connections were double-outlet right ventricle. There was a common atrium with bilateral superior caval veins and no innominate vein. There was interruption of the right-sided inferior caval vein with azygous continuation to the right superior caval vein. The hepatic veins drained separately to the floor of the right-sided atrium. The pulmonary veins drained to the nearest atrium. There was no pulmonary stenosis.

A pulmonary artery band was placed at that time, and at the age of 2 years, a right modified Blalock Taussig shunt was required. At the age of 11 years, bilateral cavopulmonary anastomoses were performed with closure of the main pulmonary artery and division of the shunt. The hepatic veins remained connected to the right-sided atrium. By 14 years of age, the child began to exhibit worsening cyanosis with arterial oxygen saturations in room air of 71%. Pulmonary arteriography demonstrated a rapid transit of contrast through the lungs compatible with pulmonary arteriovenous fistulas. This was confirmed by transesophageal echocardiography when agitated saline, injected into each pulmonary artery, showed transit of echo-contrast into the atria. A magnetic resonance image (MRI) failed to demonstrate fistulae, probably due to their small size, but it showed the entry point of the hepatic veins into the atrium and the close proximity of the azygous vein to the atrium (Fig 1).

View larger version (110K): [in this window] [in a new window]   Fig 1. Preoperative MRI showing the hepatic veins emptying into the common atrium (arrow).

View larger version (122K): [in this window] [in a new window]   Fig 2. Postoperative MRI demonstrating the hepatic veins draining into the azygous continuation of the inferior vena cava, posterior to the atrium (arrow).

Van Son and Falk have suggested the inclusion of the hepatic venous drainage in the pulmonary circulation via an extra cardiac conduit at the time of the initial repair [8]. A direct anastomosis of the hepatic veins to the azygous vein is effective in establishing hepatic drainage to the pulmonary arteries. It avoids the use of foreign material and allows for growth.

	References

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1. Cloutier A., Ash J.M., Smallhorn J.F., et al. Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure. Circulation 1985;72:471-479.[Abstract/Free Full Text]
2. Srivastava D., Preminger T., Lock J.E., et al. Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease. Circulation 1995;92:1217-1222.[Abstract/Free Full Text]
3. Kopf G.S., Laks H., Stansel H.C., Hellenbrand W.E., Kleinman C.S., Talner N.S. Thirty-year follow-up of superior vena cava-pulmonary artery (Glenn) shunts. J Thorac Cardiovasc Surg 1990;100:662-671.[Abstract]
4. Shah M.J., Rychik J., Fogel M.A., Murphy J.D., Jacobs M.L. Pulmonary AV malformations after superior cavopulmonary connection. Ann Thorac Surg 1997;63:960-963.[Abstract/Free Full Text]
5. Knight W.B., Mee R.B.B. A cure for pulmonary arteriovenous fistulas?. Ann Thorac Surg 1995;59:999-1001.[Abstract/Free Full Text]
6. Kreutzer J., Castañeda A.R., Keane J.F., Mayer J.E., Jonas R.A., Lock J.E. Surgical incorporation of hepatic veins in patients with pulmonary arteriovenous malformations (AVMs) after Kawashima operation. Cardiology in the Young 1997;7:125.
7. Lee J., Menkis A.H., Rosenberg H.C. Reversal of pulmonary arteriovenous malformation after diversion of anomalous hepatic drainage. Ann Thorac Surg 1998;65:848-849.[Abstract/Free Full Text]
8. Van Son J.A.M., Falk V. Developments in surgical palliation of univentricular physiology. Ann Thorac Surg 1997;63:301-303.[Free Full Text]

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This Article Abstract Full Text (PDF) 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 Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): David B. Ross David A. Murphy Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Baskett, R. J.F. Articles by Murphy, D. A. Search for Related Content PubMed PubMed Citation Articles by Baskett, R. J.F. Articles by Murphy, D. A.