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How To Do It

Fenestration Between the Left Atrial Appendage and Left Superior Vena Cava in Kawashima Operation

Rza Turkoz, MD^a,_*, Ouz Omay, MD^a, Canan Ayabakan, MD^b, Can Vuran, MD^a, Kurat Tokel, MD^b, lhan Günay, MD^a, Sait Alamaci, MD^a

^a Department of Cardiovascular Surgery, Baskent University, stanbul Teaching and Medical Research Center, Istanbul, Turkey
^b Department of Pediatrics, Baskent University, stanbul Teaching and Medical Research Center, Istanbul, Turkey

Accepted for publication April 25, 2008.

^_* Address correspondence to Dr Turkoz, Baskent University, Istanbul Teaching and Medical Research Center, Oymacl Sk. No: 7 Altunizade, Istanbul, 34662, Turkey (Email: rturkoz{at}yahoo.com).

	Abstract

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The benefits of a baffle fenestration in essentially high-risk Fontan patients have been demonstrated. Described here is the use of a new fenestration between the left atrial appendage and the left superior vena cava after Kawashima operation in a patient with a double-outlet right ventricle with hypoplastic left ventricle, left atrial isomerism, bilateral superior vena cavae with no bridging vein, an interrupted inferior vena cava, and continuation of the hemiazygos vein to the left superior vena cava.

	Introduction

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High-risk Fontan candidates who have the staged Fontan procedure (first bi-directional cavopulmonary anastomosis and then total cavopulmonary connection) have excellent clinical results [1]. However, patients with single ventricle, left atrial isomerism, interruption of the inferior vena cava, and bilateral superior vena cavae are often palliated with Kawashima operation. This operation consists of bilateral cavopulmonary anastomoses in which hepatic venous flow is left within the systemic circulation. Patients with a mean pulmonary arterial pressure of more than 20 mm Hg are challenging because of the risk of cavopulmonary dysfunction. The benefits of a baffle fenestration in essentially high-risk Fontan patients have been shown [2]. However, creation of a classic baffle fenestration is not technically possible during the Kawashima operation. We present the unique case of a patient with borderline mean pulmonary artery pressure who underwent a Kawashima operation and fenestration between the left atrial appendage and the left superior vena cava.

	Technique

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A cyanotic newborn was diagnosed as having left atrial isomerism, a double-outlet right ventricle, a hypoplastic left ventricle, a large ventricular septal defect, an atrial septal defect, and pulmonary stenosis. When he was 1.5 months old, he received palliative treatment at another institution with a left modified Blalock-Taussig shunt. He was 6 years old when he presented to our clinic. Cardiac catheterization confirmed the diagnosis showing bilateral superior vena cavae with no bridging vein, an interrupted inferior vena cava, and continuation of the hemiazygos vein to the left superior vena cava. The systolic blood pressure in the right ventricle was 123 mm Hg, and it was 104 mm Hg in the aorta. The mean pulmonary artery blood pressure was 22 mm Hg. The modified Blalock-Taussig shunt was patent, but was stenotic at the anastomosis site.

A Kawashima operation (bilateral cavopulmonary anastomosis) was planned, but because the mean pulmonary artery pressure was high, the operation was considered to be high risk. After standard aortic cannulation and venous cannulation through both superior vena cavae and the right atrium, cardiopulmonary bypass was begun. The main pulmonary artery and the modified Blalock-Taussig shunt were divided. The pulmonary artery branches were dissected distally and the bilateral superior vena cavae were anastomosed to the branch pulmonary arteries. A 3-mm fenestration was made using side clamps and directly anastomosing the left atrial appendage to the left superior vena cava at its cannulation site (Fig 1). After modified ultrafiltration, the pressure measurements were 75 mm Hg systolic blood pressure in the aorta, 8 to 9 mm Hg mean blood pressure in both superior vena cavae, and 4 mm Hg mean pulmonary blood pressure in the right atrium. The transpulmonary gradient was calculated as 5 mm Hg. The oxygen saturation postoperatively ranged from 80% to 86%. The bilateral chest drains were removed on day 4 after surgery, and the patient was discharged on day 8 after surgery. At 7-month follow-up, the patient is in good condition with oxygen saturation of 83%.

View larger version (18K): [in this window] [in a new window]   Fig 1. Fenestration between the left atrial appendage and the left superior vena cava for Kawashima operation in a patient with an interrupted inferior vena cava, and continuation of the hemiazygos vein to the left superior vena cava. (AO = aorta; Fen = fenestration; HA = hemiazygos vein; LAA = left atrial appendage; LSVC = left superior vena cava; PA = pulmonary artery; RA = right atrium; RSVC = right superior vena cava.)

	Comment

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Picarelli and associates [4] have done a Kawashima operation in which they make a fenestration between the right atrial appendage and the right pulmonary artery. This technique is easier if the right atrial appendage is wide and long; however, the right atrial appendage is usually situated anterior and inferior to the right pulmonary artery, making the anastomosis difficult, whereas the left atrial appendage is almost always near the left superior vena cava. There is greater risk of a pulmonary arteries stenosis when the fenestration is created directly on the pulmonary arteries during a Fontan operation. In our fenestration technique, after weaning from cardiopulmonary bypass, a direct connection is easily created by placing two side clamps on the left atrial appendage and the left superior vena cava with no risk of pulmonary artery distortion and stenosis.

In children with an interrupted inferior vena cava and azygous continuation, Kawashima operation eliminates the hepatic venous effluent from the pulmonary circulation. Consecutively this may cause pulmonary arteriovenous malformations to develop. The incidence of pulmonary arteriovenous malformations has been reported to be as high as 58% within 5 years after the Kawashima operation [5]. Brown and colleagues [5] analyzed the development of pulmonary arteriovenous malformations in their patients after the Kawashima operation. They revealed that none of their patients who had undergone completion Fontan within 18 months had evidence of pulmonary arteriovenous malformations. Furthermore, interval between Kawashima operation and completion Fontan of more than 2 years was found as the most sensitive predictor of development of pulmonary arteriovenous malformations. Therefore, as with Brown and colleagues [5], we recommend early redirection of the hepatic venous effluent to the pulmonary arterial circulation. Because transcatheter occlusion of the fenestration between the left atrial appendage and the left superior vena cava is technically difficult, redirecting the hepatic venous effluent to the pulmonary arteries and occluding the fenestration will be performed simultaneously in a second operation within 2 years of the Kawashima operation.

Creating a fenestration between the left atrial appendage and the left superior vena cava is a novel technique in a Kawashima operation. The technique is simple, quick, and without risk of a pulmonary artery stenosis and makes a reliably effective fenestration for patients with high-risk hemodynamics.

	References

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1. Masuda M, Kado H, Shiokawa Y, et al. Clinical results of the staged Fontan procedure in high-risk patients Ann Thorac Surg 1998;65:1721-1725.[Abstract/Free Full Text]
2. Bridges ND, Mayer Jr JE, Lock JE, et al. Effect of baffle fenestration on outcome of the modified Fontan operation Circulation 1992;86:1762-1769.[Abstract/Free Full Text]
3. Hannan RL, Rossi AF, Nykanen DG, et al. The fenestrated Kawashima operation for single ventricle with interrupted inferior vena cava Ann Thorac Surg 2003;75:271-273.[Abstract/Free Full Text]
4. Picarelli D, Montenegro JL, Antunez S, Perez W, Borbonet D. Modified fenestration technique for the Kawashima operation in a young infant J Thorac Cardiovasc Surg 2005;129:451-452.[Free Full Text]
5. Brown JW, Ruzmetov M, Vijay P, Rodefeld, MD, Turrentine MW. Pulmonary arteriovenous malformations in children after the Kawashima operation Ann Thorac Surg 2005;80:1592-1596.[Abstract/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): Riza Turkoz Oguz Omay Ilhan Günay Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Turkoz, R. Articles by Aslamaci, S. Search for Related Content PubMed PubMed Citation Articles by Turkoz, R. Articles by Aslamaci, S. Related Collections Congenital - acyanotic