Ann Thorac Surg 2004;77:2226-2227
© 2004 The Society of Thoracic Surgeons
How to do it
Modified cavoatriotomy for combined PAPVC repair and maze procedure
Hiroyuki Nakajima, MDa*,
Hideki Uemura, MDa,
Junjiro Kobayashi, MDa,
Koji Kagisaki, MDa,
Toshikatsu Yagihara, MDa,
Soichiro Kitamura, MDa
a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
Accepted for publication August 1, 2003.
* Address reprint requests to Dr Nakajima, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan.
e-mail: hnakajim{at}hsp.ncvc.go.jp
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Abstract
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In patients with partial anomalous pulmonary venous connection (PAPVC) to the superior cavoatrial junction, the standard right-sided left atriotomy does not allow sufficient access to the mitral valve and the left atrium. And the injury and traction of the sinus node and sinus node artery should be avoided for prevention of the cardiac rhythm disturbance after operation. We herein report a useful approach to repair the sinus venosus atrial septal defect with PAPVC of the right pulmonary veins to the superior cavoatrial junction in patients also requiring mitral valve replacement and the maze procedure.
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Introduction
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In the adult population, congenital anomalies are commonly associated with chronic atrial fibrillation (AF) [1]. Partial anomalous pulmonary venous connection (PAPVC) with sinus venosus atrial septal defect (ASD) can be a cause of AF as well as atrial arrhythmia and sinus node dysfunction before and after the surgical repair. We present a case of successful surgical treatment for a patient with PAPVC, sinus venosus ASD, and mitral stenosis combined with chronic AF.
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Technique
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A 60-year-old woman was admitted into our institution with the diagnosis of ASD, PAPVC, and mitral stenosis. She also had a one-year history of AF. Echocardiography revealed that ASD was of the sinus venosus type and that two right upper pulmonary veins were connected to the junction of the superior vena cava (SVC) and the right atrium. The mitral valve orifice area was decreased to 0.9 cm2 by sclerotic degeneration.
Surgery was carried out through a standard median sternotomy. Cardiopulmonary bypass was established with the ascending aortic and bicaval cannulations. The SVC cannula was placed at the junction of the innominate vein and SVC. Under cardiac arrest, SVC was incised longitudinally in front of the orifices of the anomalous pulmonary veins. The incision was extended through the sinus venosus ASD with continuation into a right-sided left atriotomy (Figure 1).
In the maze procedure, although only three pulmonary veins were isolated, the other maneuvers were similar to the previous report [2]. Subsequently mitral valve replacement was carried out.

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Fig 1. (A) A longitudinal incision was made in the superior vena cava (SVC) in front of the anomalous pulmonary venous orifices. The incision was extended through the sinus venosus atrial septal defect (ASD) and continued to a right-sided left atriotomy. (Dotted line = incision.) (B) A Gore-Tex patch (W. L. Gore & Associates, Flagstaff, AZ) was sutured to the wall of SVC and the rim of ASD. In addition anterosuperior advancement of the posterior rim of ASD effectively enlarged the pathway from the pulmonary veins to the left atrium. (C, D) Views from inside. (C) The orifices of the anomalous pulmonary veins and ASD had a distinctive three-dimensional relationship. The posterior rim of ASD was advanced anterosuperiorly (small arrows). (D) Shifting the plane of interatrial septum anteriorly would prevent the torsion of the patch and would be effective for prevention of stenosis of the created pathway. The arrows represent the pulmonary venous drainage without turbulence.
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The repair of PAPVC was achieved by creating a baffle with 0.1-mm thin Gore-Tex (W. L. Gore & Associates, Flagstaff, AZ) patch and concomitantly we performed an-terosuperior advancement of the posterior rim of the ASD which shifted the plane of the atrial septum anteriorly. The patient successfully regained sinus rhythm and no arrhythmia occurred for one year after these procedures.
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Comment
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ASD is one of the popular congenital anomalies in the aged population, sometimes associated with AF before and after surgical treatment [3]. Eliminating AF by the maze procedure is beneficial in terms of termination of anticoagulation and reduction of the risk of stroke [4]. In a previous report, the sinus rhythm recovery rate in patients with AF associated with ASD was satisfactory [1].
Atrial arrhythmia and sick sinus syndrome are relatively common complications after the surgical repair of PAPVC. To prevent these complications, the injury and traction of the sinus node and sinus node artery should be avoided. PAPVC repair through cavotomy without right atriotomy is effective for prevention of the postoperative rhythm disturbance [5]. However stenosis of the pulmonary venous pathway into the left atrium may occur in this procedure [5].
In the present case, we made a longitudinal incision of SVC and extended it through the sinus venosus ASD with continuation into a right-sided left atriotomy. This cavoatriotomy provided a sufficient approach to the mitral valve and the left atrium for valve replacement and the modified maze procedure [2].
This incision was also useful for the repair of PAPVC. It allowed the anterosuperior advancement of the posterior rim of ASD which effectively enlarged the pathway from the pulmonary veins into the left atrium via ASD by shifting the plane of the interatrial septum anteriorly. In addition the Gore-Tex patch was sutured to the rim of ASD and the SVC wall and as the blood supply to the sinus node was intact, the risk of rhythm disturbance after this procedure of PAPVC repair would be considered minimal.
Our modified cavoatriotomy provided an excellent access to the mitral valve and the left atrium. This incision was useful for the simultaneous PAPVC repair and advantageous in sinus rhythm restoration. The maze procedure is applicable and effective for AF associated with PAPVC.
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References
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- Kobayashi J., Yamamoto F., Nakano K., Sasako Y., Kitamura S., Kosakai Y. Maze procedure for atrial fibrillation associated with atrial septal defect. Circulation 1998;98:II-399-402.
- Nakajima H., Kobayashi J., Bando K., et al. The effect of cryo-maze procedure on early and intermediate term outcome in mitral valve disease. Circulation 2002;106(Suppl I):I-46-50.
- Gatzoulis M.A., Freeman M.A., Siu S.C., Webb G.D., Harris L. Atrial arhythmia after closure of atrial septal defects in adults. N Engl J Med 1999;340:839-846.[Abstract/Free Full Text]
- Cox J.L., Jaquiss R.D.B., Schuessler R.B., Boineau J.P. Modification of the maze procedure for atrial flutter and atrial fibrillation. J Thorac Cardiovasc Surg 1995;110:485-495.[Abstract/Free Full Text]
- Nicholson I.A., Chard R.B., Nunn G.R., Cartmill T.B. Transcaval repair of the sinus venosus syndrome. J Thorac Cardiovasc Surg 2000;119:741-744.[Abstract/Free Full Text]