Eur J Cardiothorac Surg 2002;21:582-584
© 2002 Elsevier Science NL
Hepatic vein-to-azygos vein connection in a patient with interrupted inferior vena cava
Y. Kaneko*,
A. Murakami,
T. Miyamoto,
S. Takamoto
Department of Cardiothoracic Surgery, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan
Received 10 September 2001;
received in revised form 28 November 2001;
accepted 20 December 2001.
* Corresponding author. Department of Cardiovascular Surgery, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150-8935, Japan. Tel.: +81-3-3400-1311; fax: +81-3-3409-1604
e-mail: yukihirokaneko{at}hotmail.com
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Abstract
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Massive systemic-to-hepatic venous collaterals developed in a 8-year-old boy with left isomerism and azygos connection of the inferior vena cava who had undergone total cavopulmonary shunt leaving the hepatic vein connected to the common atrium 5 years previously. A hepatic vein-to-azygos vein connection via an autologous pericardial roll successfully ameliorated cyanosis and physical activity. Hepatic vein-to-azygos vein connection is technically easy and is favorable in providing balanced distribution of hepatic venous blood to both lungs. We recommend this technique as an effective alternative to hepatic vein-to-pulmonary artery connection in patients developing collateral vessels after total cavopulmonary shunt.
Key Words: Fontan procedure/adverse effect • Heart atrium • Heart defect, congenital/surgery • Postoperative complications • Reoperation
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1. Introduction
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In patients with azygos connection of the inferior vena cava (IVC), total cavopulmonary shunt (TCPS) leaving the hepatic vein connected to the atrium predisposes collateral vessel formation from systemic veins to the portal or hepatic vein and from the pulmonary artery to the pulmonary vein. Progressive cyanosis caused by shunt through the collateral vessels warrants surgical inclusion of the hepatic vein in pulmonary circulation [1]. This report describes hepatic vein-to-azygos vein connection instead of hepatic vein-to-pulmonary artery connection for inclusion of the hepatic vein in pulmonary circulation in a patient after TCPS.
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2. Case report
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A cyanosed newborn male was diagnosed as having left isomerism, single atrium, single right ventricle with left-hand topology, dextrocardia, pulmonary atresia, and right-sided azygos connection of the IVC to the single right-sided superior vena cava (SVC). An original left Blalock–Taussig shunt was created at 2 months of age. At 3 years of age, TCPS was established. The SVC was transected below the azygos vein, and its cranial end was anastomosed to an incision in the superior surface of the central pulmonary artery with its caudal end oversewn. By 8 years of age, he exhibited deteriorating physical activity and cyanosis. Oxymetry demonstrated arterial, pulmonary venous, and mixed venous oxygen saturations respectively of 78.7, 90.1, and 68.1%, indicating that 52% of systemic venous return drains through the hepatic vein bypassing pulmonary circulation. Computed tomogram demonstrated two hepatic veins separately connected to the atrium.
Through a re-sternotomy and an incision in the right groin, cardiopulmonary bypass was established with an aortic cannula and four venous cannulas placed in the SVC, right femoral vein, and both hepatic veins. Identification of the azygos vein in the posterior mediastinum was facilitated by palpating the femoral venous cannula that advanced into the azygos vein. During aortic cross-clamping with continuous aortic root perfusion, an atrial cuff including the hepatic veins was excised from the atrium followed by suture closure of the atrial wall defect. The cuff was connected to the azygos vein via an interposing autologous pericardial roll (Fig. 1)
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Fig. 1. The surgical technique of hepatic vein-to-azygos vein connection. (Left) Intraoperative view of the heart and great vessels. (Middle) An atrial cuff including the hepatic veins is excised from the common atrium, and the azygos vein, vagal nerve, and esophagus in the posterior mediastinum are exposed. A double-headed arrow indicates the incision in the azygos vein. (Right) The azygos vein and atrial cuff are being anastomosed to an autologous pericardial roll. Az, azygos vein; E, esophagus; H, hepatic vein; V, vagal nerve.
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Postoperatively, the patient experienced transient liver dysfunction with serum aspartate aminotransferase and alanine aminotransferase respectively elevated to 1135 and 1126 IU/l on day 2, both of which normalized within 2 weeks. He was intubated for 4 days and stayed in an intensive care unit for 5 days. The postoperative course was otherwise uneventful. Oral aspirin of 150 mg was administered postoperatively as antiplatelet therapy. Normal blood ammonia level, signs, and symptoms ruled out possible portal-systemic encephalopathy caused by reversed shunt from the portal to systemic vein. An angiogram revealed unobstructed hepatic venous drainage with caval pressure of 19 mmHg (Fig. 2) . Arterial oxygen saturation was 93.8% in room air. He enjoys improved physical activity 8 months after surgery.
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Fig. 2. Lateral view of the postoperative hepatic venous angiogram shows unobstructive hepatic vein-to-azygos vein connection. A bracket indicates an interposing autologous pericardial roll.
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3. Comment
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In our institution, TCPS has been established in five patients at ages from 1 to 5 years (mean 3.6 years) with left isomerism and azygos connection of the IVC. Cyanosis caused by collateral vessel formation warranted inclusion of the hepatic vein in pulmonary circulation in three patients from 5 to 13 years after TCPS. One patient was reported herein, and the other two patients underwent hepatic vein-to-pulmonary artery connection via an extra-cardiac expanded polytetrafluoroethylene conduit.
The present case is the second reported case of hepatic vein-to azygos vein connection next to the case reported by Baskett et al. [2]. Except for these two cases, hepatic vein-to-pulmonary artery connection has been exclusively performed for the purpose of including the hepatic vein in pulmonary circulation after TCPS [1,3,4]. Hepatic vein-to-pulmonary artery connection entails exposure of the pulmonary artery surrounded by a previous surgical scar, which may be technically demanding. In addition, individual surgical techniques for hepatic vein-to-pulmonary artery connection, namely the intra-atrial lateral tunnel technique, intra-atrial conduit technique and extra-cardiac conduit technique, have their inherent drawbacks similar to corresponding techniques as a part of the Fontan procedure. The intra-atrial lateral tunnel technique may cause atrial arrhythmia because of a long atrial incision/suture line. The intra-atrial conduit technique entails atrial incision and has a potential disadvantage of conduit stenosis and systemic thromboembolism. The extra-cardiac conduit technique entails extensive mobilization of intra-pericardial adhesion for proper placement of the graft, and has a potential risk of graft stenosis/occlusion.
We adopted hepatic vein-to-azygos vein connection as the preferred surgical technique in this patient for two reasons as described below. Firstly, exposure of the azygos vein, being free of any previous surgical scar, is technically easier than exposure of the pulmonary artery. Assumedly, therefore, hepatic vein-to-azygos vein connection is more comfortable to the surgeon and is associated with a lower risk of phrenic nerve injury than hepatic vein-to-pulmonary artery connection. Secondly, distribution of hepatic venous blood is balanced in the bilateral lungs after hepatic vein-to-azygos vein connection. Hepatic venous blood is believed to contain a putative factor that prohibits pulmonary collateral vessel development [5]. Balanced distribution of hepatic venous blood to bilateral lungs, being favorable to resolution of pulmonary collateral vessels, would be difficult to attain by hepatic vein-to-pulmonary artery connection without causing turbulent blood flow in patients with single SVC, particularly when TCPS was previously placed behind the systemic ventricle [3].
In the case report of hepatic vein-to-azygos vein connection by Baskett et al. [2], direct anastomosis between the hepatic vein and azygos vein was made using deep hypothermia and circulatory arrest, whereas we employed interposition of a pericardial roll because of remote disposition of the hepatic veins and azygos vein. Our impression is that hepatic vein-to-azygos vein connection can be made without circulatory arrest as long as an interposing conduit can be used. The fact that a considerable proportion of patients after TCPS suffer from deteriorating cyanosis justifies the inclusion of the hepatic vein in pulmonary circulation at the time of TCPS to prevent later collateral vessel formation [5,6]. In case of collateral vessel formation after TCPS with the hepatic venous drainage bypassing the pulmonary circulation, hepatic vein-to-azygos vein connection can be an effective alternative to hepatic vein-to-pulmonary artery connection for resolution of cyanosis.
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Abstract
1. Introduction
