Ann Thorac Surg 1998;65:1783-1784
© 1998 The Society of Thoracic Surgeons
Case Reports
Anastomosis of the Left Juxtaposed Atrial Appendages in a Patient With Tricuspid Atresia
Yukihiro Kaneko, MDa,
Hideo Okabe, MDa,
Nobuhiro Nagata, MDa,
Jotaro Kobayashi, MDa,
Shinya Kanemoto, MDa
a Department of Thoracic and Cardiovascular Surgery, Kanagawa Children’s Medical Center, Yokohama, Japan
Accepted for publication December 29, 1997.
Address reprint requests to Dr Kaneko, Department of Cardiothoracic Surgery, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113, Japan
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Abstract
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A 9-month-old boy with left juxtaposition of the atrial appendages, tricuspid atresia, pulmonary atresia, and ventriculoarterial discordance underwent anastomosis between the atrial appendages after failure of balloon/blade atrial septostomy because of restrictive atrial septal defect. For surgical creation of atrial communication in patients with juxtaposed atrial appendages, anastomosis between the atrial appendages seemed to be safer, more effective, and less invasive than septectomy by Blalock-Hanlon technique or inflow occlusion technique.
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Introduction
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At 32 weeks’ gestation, a fetal echocardiogram revealed tricuspid atresia and complete atrioventricular block. Transthoracic echocardiogram after cesarean delivery demonstrated pulmonary atresia, ventriculoarterial discordance, a nonobstructive atrial septal defect measuring 7 mm, and right aortic arch with mirror-image branching; echocardiography failed to detect left juxtaposition of the atrial appendages (LJAA). A DDD pacemaker with epicardial electrodes was implanted and a prosthetic left subclavian artery-to-pulmonary artery shunt was placed when the infant was 6 days of age, but the baby exhibited impaired weight gain thereafter. When the patient was 9 months of age with a body weight of 4 kg, systemic venous congestion due to restrictive atrial communication developed along with deterioration in cyanosis. An echocardiogram confirmed the absence of subaortic obstruction. Balloon/blade atrial septostomy under fluoroscopy was attempted but was ineffective. During the attempts of septostomy, a right atrial angiogram revealed LJAA (Fig 1). A pulmonary arterial angiogram showed left pulmonary artery hypoplasia. Because the patient had low body weight and left pulmonary artery hypoplasia, surgical creation of atrial communication and placement of an additional systemic to pulmonary shunt were warranted rather than bidirectional cavopulmonary connection or a Fontan-type procedure.
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Fig 1. Right atrial cineangiogram in diastole. The right atrial appendage (black dotted line), being located to the left of the aorta (Ao), lies adjacent to the left atrial appendage (white dotted line). (LV = left ventricle; RA = body of the right atrium.)
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At operation, the heart was exposed via a median sternotomy. As sufficient oxygenation was maintained during clamping of the left brachiocephalic artery, a prosthetic shunt was created between the left brachiocephalic artery and the left pulmonary artery with the patient in a stable condition. Intraoperative pressure measurements of both atria revealed a mean pressure gradient of 5 mm Hg. Each atrial appendage was excluded by a curved vascular clamp and an ample anastomosis was made between them with an absorbable suture. Right atrial pressure decreased from 13 mm Hg to 8 mm Hg, and pressures in both atria equalized after this procedure. Postoperatively, systemic venous congestion and cyanosis were ameliorated. A transthoracic echocardiogram demonstrated the anastomosis between the atrial appendages to be 13 mm in diameter with no detectable pressure gradient. The patient was discharged on postoperative day 14, and is awaiting a Fontan-type procedure.
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Comment
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Left juxtaposition of the atrial appendages is a rare condition in which both atrial appendages lie to the left of the great arteries. Left juxtaposition of the atrial appendages frequently coexists with tricuspid valve anomaly and ventriculoarterial discordance. The incidence of LJAA in infants with tricuspid atresia was reported to be about 10%, and especially when ventriculoarterial discordance was also present, the incidence was as high as 30% [1].
The presence of adequate atrial communication is indispensable to a patient with tricuspid atresia until the patient is old enough to undergo a Fontan-type procedure. Such a communication, usually present at birth, will often become restrictive with increasing age and a sufficient communication will then have to be created. Balloon/blade atrial septostomy is usually successful in neonates, but is sometimes ineffective after they reach 3 months of age because of thickening of the atrial septum [2]. Consequently, surgical septectomy is necessary in selected infants even in the current era.
Because LJAA highly correlates with ventriculoarterial discordance, and ventriculoarterial discordance predisposes to restrictive atrial communication, creation of an atrial communication is more frequently necessary in patients with LJAA than in those without LJAA [3]. Moreover, because the atrial septum is transversely oriented and is smaller than normal in hearts with LJAA, balloon/blade atrial septostomy is known to be more difficult and carries a higher risk of rupture at the right atrial/caval vein junction [4]. For these reasons, patients with tricuspid atresia and LJAA undergo surgical creation of an atrial communication more frequently than those without LJAA.
Currently, surgical septectomy including Blalock-Hanlon technique and inflow occlusion technique are infrequently performed, and outcomes are rarely reported. However, these operations are still considered to carry considerable risk [5]. Wood and associates [6] reported that Blalock-Hanlon septectomy is particularly difficult to perform in the presence of LJAA. The narrow free right atrial wall makes it difficult to apply a clamp while allowing an adequate amount of septum to be resected. Because the septum is small, the surgeon must avoid resecting an excessive amount of tissue, which would cause life-threatening exsanguination. Atrial septectomy under inflow occlusion is also hazardous in the presence of LJAA. As viewed from a right atriotomy, the orifice of the right atrial appendage looks similar to the unusually located septal defect, although it is more anterior. If a hurried surgeon mistook this orifice for the septal defect to be enlarged, disastrous results would follow [1].
As an alternative to septectomy in patients with LJAA, anastomosis of the atrial appendages was first reported by Moene and Brom [7] in a patient with transposition of the great arteries. After their report, 6 attempts at Blalock-Hanlon procedure were reported in patients with LJAA [6, 8]. In these reports, there was no reference to anastomosis of the atrial appendages as a possible option. To our knowledge, this is the second report of anastomosis of the atrial appendages instead of septectomy. The atrial communication created by the procedure was wide enough to accommodate systemic venous return freely. We consider this method safe and effective; the operation can be carried out without destabilizing the hemodynamic status or causing pulmonary congestion, and the procedure is technically easy and has virtually no time limits. We recommend anastomosis of the atrial appendages as the first-choice technique for surgical creation of an atrial communication in patients with LJAA.
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References
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- Thoele D.G., Ursell P.C., Ho S.Y., et al. Atrial morphologic features in tricuspid atresia. J Thorac Cardiovasc Surg 1991;102:606-610.[Abstract]
- Webber S.A., Culham J.A.G., Sandor G.G.S., Patterson M.W.H. Balloon dilatation of restrictive interatrial communications in congenital heart disease. Br Heart J 1991;65:346-348.[Abstract/Free Full Text]
- Weinberg P.M. Anatomy of tricuspid atresia and its relevance to current form of surgical therapy. Ann Thorac Surg 1980;29:306-311.[Abstract/Free Full Text]
- Anjos R.T., Ho S.Y., Anderson R.H. Surgical implications of juxtaposition of the atrial appendages. J Thorac Cardiovasc Surg 1990;99:897-904.[Abstract]
- Stark J. Surgical septectomy. In: Stark J., de Leval M., eds. Surgery for congenital heart disease, 2nd ed. Philadelphia: Saunders, 1994:269-274.
- Wood A.E., Freedom R.M., Williams W.G., Trusler G.A. The Mustard procedure in transposition of the great arteries associated with juxtaposition of the atrial appendages with and without dextrocardia. J Thorac Cardiovasc Surg 1983;85:451-456.[Abstract]
- Moene R.J., Brom A.G. Anastomosis between the atrial appendages in a patient with juxtaposition. Chest 1971;59:583-586.[Abstract/Free Full Text]
- Urban A.E., Stark J., Waterston D.J. Mustard’s operation for transposition of the great arteries complicated by juxtaposition of the atrial appendages. Ann Thorac Surg 1976;21:304-310.[Abstract/Free Full Text]
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Abstract
Introduction
