Ann Thorac Surg 1999;67:260-262
© 1999 The Society of Thoracic Surgeons
How To Do It
Technique for repair of single-ventricle hearts with transposition of the great arteries and aortic arch hypoplasia
Patricia A. Thistlewaite, MD, PhDa,
John L. Myers, MDa,
Ralph D. Siewers, MDa,
José A. Ettedgui, MDa
a Divisions of Cardiothoracic Surgery and Pediatric Cardiology, University of Pittsburgh School of Medicine, The Children’s Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA
Accepted for publication June 30, 1998.
Address reprint requests to Dr. Myers, Pediatric and Congenital Cardiovascular Surgery, Children’s Hospital, Milton S. Hershey Medical Center, MC H165, Box 850, Hershey, PA 17033
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Abstract
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We describe a modified technique of aortopulmonary anastomosis for palliative repair of hearts with a single left ventricle, a rudimentary right ventricle, transposition of the great vessels, and a hypoplastic aortic arch. This procedure creates an unobstructed left ventricular outflow tract and avoids the problems of spiral patch grafting and multiple patch enlargements of the ascending aorta and arch.
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Introduction
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The Norwood operation has provided a reliable method for initial palliation of infants with hypoplastic left heart syndrome and normally related great arteries. Some infants with hypoplastic aortic arch and single ventricle anatomy have more complex great vessel and ventricular relationships and require some modification of the operation that Norwood developed. This article describes a modification of the Norwood operation which we have found useful in these patients with complex great vessel and ventricular relationships.
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Materials and methods
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Three patients with single-ventricle hearts with transposition of the great arteries, a rudimentary right ventricle, and a hypoplastic aortic arch were managed by a technique that is a modification of the Norwood operation. Two patients had situs solitus, atrioventricular discordance, and ventricular arterial discordance (Fig 1) and 1 had situs solitus, atrioventricular concordance, and ventricular arterial discordance cardiac morphology with tricuspid atresia.
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Fig 1. Heart with single-ventricle anatomy, situ solitus, atrioventricular discordance, and ventricular arterial discordance, double-inlet left ventricle, hypoplasia, and coarctation of the aorta. Dashed lines illustrate surgical incisions.
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These neonates underwent a palliative repair using a modification of the Norwood technique (Fig 2). Through a median sternotomy approach, the ascending aorta and main pulmonary artery were both cannulated because there was significant distal aortic hypoplasia, and a single venous cannula was placed in the right atrial appendage. If ascending aortic hypoplasia is significant and there is retrograde perfusion of the aortic arch and ascending aorta, then arterial cannulation is accomplished by a single cannula in the main pulmonary artery. After cardiopulmonary bypass was instituted, both pulmonary artery branches were rapidly occluded. The infants were cooled during bypass for 20 to 30 minutes to 16°C, during which time the innominate, left carotid, and left subclavian arteries were exposed and encircled with tourniquets. After circulatory arrest was established and the head vessels were occluded, the main pulmonary artery and ascending aorta were transected proximally, leaving the proximal ascending aorta approximately 5 to 10 mm longer than the proximal pulmonary artery. An incision in the left lateral side of the distal ascending aorta was extended along the inner aspect of the arch to beyond the ductus arteriosus. After excising all ductal tissue, a patch of pulmonary allograft was sewn into the aorta, gusseting the upper descending aorta, transverse arch, and ascending aorta (Fig 2, inset). Native ascending aorta makes up a portion of the circumference of the new ascending aorta, thus allowing for growth potential. The enlarged and reconstructed aortic arch was then anastomosed end to end to the proximal pulmonary trunk that arose from the left ventricle (Fig 3). Anterior and to the left and within the pulmonary–aortic anastomosis, an oval defect was created (Fig 3, inset). The proximal ascending aorta, arising from the outflow chamber, was beveled slightly and anastomosed to this opening. One half of this anastomosis is to native pulmonary artery and the superior half to the pulmonary allograft. We believe that this will provide growth potential. Cardiopulmonary bypass was reestablished, and the distal main pulmonary artery was closed with a patch of the pulmonary homograft. A 3.5- or 4.0-mm polytetrafluoroethylene shunt was placed between the innominate artery and the right pulmonary artery.
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Fig 2. The main pulmonary artery is transected just proximal to the pulmonary artery confluence. The ascending aorta is transected 1 cm longer. After ligation and division of the ductus arteriosus, the hypoplastic aorta is incised along its inner curvature from 1 cm distal to the transected ductus arteriosus to the proximally transected ascending aorta. Pulmonary patch homograft augments the neoaorta (inset).
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Fig 3. Completed repair with systemic-to-pulmonary artery polytetrafluoroethylene shunt. Transfected end of ascending aorta is beveled and sewn end to side into the aortopulmonary anastomosis (inset).
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Results
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Transthoracic echocardiograms obtained postoperatively demonstrated no gradient from the systemic left ventricle to the ascending neoaorta. Follow-up cardiac catheterization has been performed in all 3 patients at 1 year postoperatively and confirmed a patent two-coronary system with retrograde and antegrade perfusion of the aortic root. All 3 patients have subsequently undergone a successful hemi-Fontan procedure.
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Comment
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The univentricular heart with transposition of the aorta arising from a rudimentary right ventricle and coexisting with aortic arch hypoplasia and coarctation of the aorta is an anatomic condition with potential systemic arterial obstruction at several levels: the bulboventricular foramen or ventricular septal defect, the aortic annulus, the aortic arch, and the distal aorta at the level of insertion of the ductus arteriosus. The goal of surgical palliation using a modified Norwood approach is to eliminate all levels of obstruction to systemic blood flow and to provide controlled pulmonary blood flow that will allow pulmonary artery growth and avoid pulmonary overcirculation.
The modified Norwood technique we describe results in a wide unobstructed connection between the functional single ventricle of left morphology and the systemic circulation. It uses the native ascending aorta, the main pulmonary artery, and a pulmonary allograft patch to augment the aortic isthmus, arch, and pulmonary–aortic anastomosis. Several other techniques have been used to reconstruct the aortic arch in patients with abnormal spatial relationships of the great arteries [1–3]. These included a common channel aortopulmonary trunk technique, in which the proximal pulmonary artery and aorta are sewn together side to side as one unit to create the base of the neoaorta [1]. This method requires a wide pulmonary patch allograft extending circumferentially around the distal aortic root. A second surgical method has been reported in which two aortic incisions are made 180 degrees apart from one another, and patches of allograft are used to connect the pulmonary artery, aorta, and pulmonary allograft to form a functional outflow conduit [3]. Both of these techniques may lead to a geometric distortion of the proximal pulmonary–aortic trunk and potentially produce valvular insufficiency.
The method we have described avoids four potential technical pitfalls: (1) a spiraling incision in the aorta from the inner curvature of the aortic arch to the posterolateral side of the ascending aorta, (2) multiple incisions in the ascending aorta, (3) circumferential allografts, and (4) potential torsion of the aortic root, resulting in obstruction to the coronary circulation and aortic or pulmonary valve insufficiency, or both.
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References
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van Son J.A., Reddy V.M., Haas G.S., Hanley F. Modified surgical techniques for relief of aortic obstruction in {S, L, L} hearts with rudimentary right ventricle and restrictive bulboventricular foramen. J Thorac Cardiovasc Surg 1995;110:909-915.[Abstract/Free Full Text]
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Rychik J., Murdison K.A., Chin A.J., Norwood W.F. Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis. J Am Coll Cardiol 1991;18:809-816.[Abstract]
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Jacobs M.L., Rychik J., Murphy J.D., Nicholson S.C., Steven J.M., Norwood W. Results of Norwood operation for lesions other than hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 1995;110:1535-1562.
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Abstract
Introduction
