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Ann Thorac Surg 2003;75:1640-1642
© 2003 The Society of Thoracic Surgeons

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Case report

New approach to the surgical management of pulmonary arteriovenous malformations after cavopulmonary anastomosis

^a Departments of Cardiovascular Surgery and Pediatric Cardiology, SUNY Upstate Medical University, Syracuse, New York, USA

Accepted for publication November 13, 2002.

^* Address reprint requests to Dr Steinberg, Department of Surgery, SUNY Upstate Medical University, 750 East Adams St, Syracuse, NY 13210, USA
e-mail: steinbja{at}upstate.edu

	Abstract

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The development of pulmonary arteriovenous malformations after cavopulmonary bypass in patients with congenital heart disease is well documented. We report successful management of pulmonary arteriovenous malformations after cavopulmonary bypass in a patient with an interrupted inferior vena cava (IVC) and multiple hepatic veins utilizing an extracardiac conduit from the hepatic veins to the hemiazygous continuation of the interrupted IVC. This technique, performed without circulatory arrest or an atriotomy, may limit morbidity associated with intracardiac procedures in patients with single ventricle morphology. Furthermore, this case suggests an alternative technique for completion Fontan in patients with an interrupted IVC and multiple hepatic venous drainage.

	Introduction

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Long-term evaluation after bidirectional cavopulmonary anastomosis (BDCPA) has revealed the development of pulmonary arteriovenous malformations (PAVMs), particularly in patients with an interrupted inferior vena cava (IVC) [1–3]. The exclusion of hepatic venous return is theorized as the causative factor, as evidenced by successful surgical management after redirection of hepatic venous flow to the pulmonary circulation via an intra-atrial lateral tunnel [3–5]. This option, however, carries morbidity related to circulatory arrest, global myocardial ischemia to the single ventricle, and an atriotomy with its potential for dysrhythmias. It has been suggested that an extracardiac conduit between confluent hepatic veins and the pulmonary arteries may improve the early and late outcomes of patients with an interrupted IVC [1]. However, in a patient with multiple hepatic veins, this conduit may be technically more difficult to construct, and an alternative type of extracardiac conduit may be more feasible.

A 16-year-old boy was born with dextrocardia, bilateral superior vena cava (SVC), interrupted left IVC with hemiazygous continuation to the left SVC, and hypoplastic left heart. At 9 months, a Blalock-Taussig shunt was performed, and at 6 years of age, the patient underwent a bilateral bidirectional Glenn procedure. Subsequent serial follow-up revealed an asymptomatic child developing normally with pulse oximetry measuring 90% to 92%. No further operation was planned to include hepatic venous flow to the pulmonary circulation because the child progressed normally without symptoms.

By the age of 13 years, however, the patient began complaining of shortness of breath and had worsening cyanosis. Pulse oximetry measured 77% and hemoglobin measured 17.6 g%. Transthoracic echocardiogram with agitated saline contrast revealed intrapulmonary shunting consistent with PAVMs. Cardiac catheterization revealed a mean pulmonary artery pressure of 11 mm Hg and ventricular end-diastolic pressure of 8 mm Hg. Mixed venous saturation measured 59%, systemic arterial saturation 81%, and PaO₂ 52 mm Hg. Pulmonary angiography revealed prompt filling of the pulmonary veins, confirming the presence of bilateral PAVMs.

The patient was taken to the operating room and through a median sternotomy, with normothermic cardiopulmonary bypass (with cannulation of the left SVC and the aorta), a 12-mm polytetrafluoroethylene (PTFE) conduit was placed between the hepatic veins and the hemiazygous continuation of the IVC (Fig 1A). Operative findings included multiple hepatic veins with right and left hepatic veins draining into the common atrium at separate locations. The patient tolerated the procedure well with no complications. At discharge (8 days later), pulse oximetery measured 93%. Cardiac catheterization 29 months later revealed significant improvement in mixed venous saturation to 77%, systemic arterial saturation to 94%, and PaO₂ to 71 mm Hg, and no change in the hemodynamic data. Repeat pulmonary angiogram and contrast echocardiogram demonstrated no residual pulmonary arteriovenous malformations in the right lung and significantly decreased shunting in the left lung, as well as patency of both hepatic vein to conduit and conduit to hemiazygous vein anastomoses (Fig 1B). The patient was maintained on coumadin for 29 months after discharge.

View larger version (21K): [in this window] [in a new window]   Fig 1. (A) Placement of polytetrafluoroethylene conduit between the hepatic veins and the hemiazygous continuation of the inferior vena cava. (H = hemiazygous vein; LHV = left hepatic vein; LSVC = left superior vena cava; RHV = right hepatic vein; RSVC = right superior vena cava.) (B) Postoperative angiogram demonstrating patency of all anastomoses 29 months after operation.

	Comment

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Potential limitations of our technique include the need for anticoagulation due to the use of an extracardiac prosthetic venous conduit and the type of anastomosis performed. Regarding the use of coumadin in our patient, we would argue that anticoagulation after Fontan procedure remains controversial, and is routinely used in patients after intraatrial lateral tunnel construction as well to prevent intracardiac venous thrombosis [7]. Additionally, although we would submit the construction of end-to-side anastomoses in our graft might lead to more turbulent flow, we believe our patient’s anatomic variation of hepatic venous drainage made this necessary to more easily construct an extracardiac bypass conduit to the pulmonary circulation.

Therefore, we believe our technique should be considered in the treatment of PAVMs that develop after BDCPA in patients with an interrupted IVC and return of multiple hepatic veins to the atrium because it avoids the morbidity associated with an intracardiac operation after single-ventricle surgery. Furthermore, support of this method is illustrated in a recent report from Baskett and associates [8], who describe the successful treatment of PAVM’s with redirection of hepatic venous flow to the pulmonary circulation through an "extracardiac" conduit to the azygos vein. However, this "extracardiac" technique involved an atrial incision and circulatory arrest, whereas ours does not. In conclusion, we postulate that incorporation of our procedure at the time of BDCPA should be considered in this particular complex patient population to prevent the formation of PAVMs.

	Acknowledgments

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We thank May Thao for design of the illustration.

	References

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1. Cloutier A., Ash J., Smallhorn J., et al. Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistulae. Circulation 1985;72:471-479.[Abstract/Free Full Text]
2. Kopf G., Laks H., Stansel H., Hellenbrand W., Kleinman C., Talner N. Thirty-year follow-up of superior vena cava-pulmonary artery shunts. J Thorac Cardiovasc Surg 1990;100:662-671.[Abstract]
3. Shah M., Rychik J., Fogel M., Murphy J., Jacobs M. Pulmonary AV malformations after superior cavopulmoanry connection: resolution after inclusion of hepatic veins in the pulmonary circulation. Ann Thorac Surg 1997;63:60-63.
4. Srivastava D., Preminger T., Lock J., 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]
5. Lee J., Menkis A., Rosenberg H. Reversal of pulmonary arteriovenous malformation after diversion of anomalous hepatic drainage. Ann Thorac Surg 1998;65:848-849.[Abstract/Free Full Text]
6. Uemura H., Yagihara T., Hattori R., et al. Redirection of hepatic venous drainage after total cavopulmonary shunt in left isomerism. Ann Thorac Surg 1999;68:1731-1735.[Abstract/Free Full Text]
7. Monagle P., Cochrane A., McCrindle B., et al. Thrmboembolic complications after Fontan procedure: the role of prophylactic anticoagulation. J Thorac Cardiovasc Surg 1998;115:493-498.[Free Full Text]
8. Baskett R., Ross D., Warren A., Sharratt G., Murphy D. Hepatic vein to the azygous vein anastomosis for pulmonary arteriovenous fistulae. Ann horac Surg 1999;68:232-233.

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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): George M. Alfieris Berkeley Brandt, III Gregory W. Fink Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Steinberg, J. Articles by Halter, J. Search for Related Content PubMed PubMed Citation Articles by Steinberg, J. Articles by Halter, J. Related Collections Congenital - cyanotic