Eur J Cardiothorac Surg 2000;17:85-87
© 2000 Elsevier Science NL
Repair of aortic atresia and hypoplastic left heart syndrome without using graft material
Zsolt L. Nagy,
Jonathan M. Parsons,
Kevin G. Watterson
Yorkshire Heart Centre, Calverley St, Leeds LS 1 3EX, UK
Corresponding author. Tel.: +44-113-392-5738; fax: +44-113-392-5865
e-mail: kevingw{at}ulth.uk
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Abstract
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We present a modification of the Norwood stage 1 operation, where the neo-aorta was reconstructed without using graft material. After extensive mobilization of the descending aorta the ductus arteriosus was excised and an end-to-end anastomosis was created between the main pulmonary artery and the distal ductal–aortic junction. The proximal ascending aorta was anastomosed side-to-side to the neo-aorta. Finally either a direct side-to-side anastomosis was created or a 3.5-mm Gore-Tex graft was implanted between the innominate artery and the right pulmonary artery. The first three patients had a favourable outcome: echocardiography showed good ventricular function and acceptable saturation (85%) at the follow up.
Key Words: Aortic atresia • Hypoplastic left heart syndrome • Modified surgical correction
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1. Introduction
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We report a modification of the Norwood stage 1 palliation, where no graft material was used for reconstruction of the neo-aorta. The procedure was performed in three consecutive patients presenting with aortic atresia. All three patients survived the initial operation and are awaiting the second stage in good condition.
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2. Surgical technique
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Median sternotomy was performed. The arterial cannula was placed in the ductus arteriosus, venous return from the right atrium via a single venous cannula. Deep hypothermia and circulatory arrest was used. The brachiocephalic vessels and the descending aorta were mobilized extensively during the cooling phase. The large main pulmonary artery was completely freed from the tiny ascending aorta (Fig. 1a). The distal transverse arch and the innominate artery were then clamped and cold crystalloid cardioplegia was given through the proximal transverse arch. The circulation was arrested and the clamps were removed. The main pulmonary artery was divided and the branch pulmonary arteries were disconnected from the main pulmonary artery with a flap of vessel wall in such a way that anteriorly the remnant of the main pulmonary artery (neo-aorta) wall was longer than posteriorly. Then the ductus arteriosus was ligated proximally, the distal end was disconnected from the descending aorta (Fig. 1b). The descending aorta was mobilized further until it reached the proximal main pulmonary artery comfortably, without compressing the left pulmonary artery or the left main bronchus. The proximal main pulmonary artery was directly anastomosed to the distal ductal–aortic junction using a 7/0 polypropylene suture. Thereafter, a generous side-to-side anastomosis was created between the neo-aorta and the tiny ascending aorta just above the valve level. The ascending aorta was then ligated above the anastomosis. The neo-aorta was cannulated, cardiopulmonary bypass recommenced. During rewarming the continuity of the pulmonary artery branches was reconstructed using autologous pericardium. Finally, the distal remnant of the tiny ascending aorta was disconnected from the transverse arch and the remaining hole was enlarged to a 3.5–4.0-mm diameter towards the innominate artery. An incision was made on the superior edge of the right pulmonary artery and either a direct side-to-side anastomosis was easily created between the two vessels without distorting them (Fig. 1c), or a 3.5-mm Gore-Tex tubular graft was implanted. The cardiopulmonary bypass was discontinued and the skin was closed.
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Fig. 1. (a) The head vessels, pulmonary arteries and descending aorta were mobilized, the main pulmonary artery was freed from the ascending aorta. (b). The branch pulmonary arteries were disconnected from the main pulmonary artery and reconstructed using pericardium patch. The ductus arteriosus was ligated proximally, the distal end was disconnected from the descending aorta. (c) The main pulmonary artery was anastomosed to the distal ductal–aortic junction. A side-to-side anastomosis was created between the proximal ascending aorta and the neo-aorta. A direct side-to-side anastomosis was constructed between the innominate artery and the right pulmonary artery.
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3. Comment
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Aortic atresia as a part of the hypoplastic left heart syndrome is the fourth commonest congenital cardiac anomaly, accounting for about 7.5% of all congenital heart disease [1]. The complete morphologic features and diagnostic criteria of the hypoplastic left heart syndrome were first described by Noonan and Nadas in 1958 [2]. Despite increasing knowledge about the pathophysiology of the condition, surgical treatment remained unsuccessful until 1983, when Norwood and co-workers reported a successful two-stage correction [3]. Many surgeons accepted the technique and the Norwood procedure became the standard approach for repairing aortic atresia. Although the results of the first stage palliation are continuously improving, there is still a significant morbidity and mortality related to the homograft reconstruction of the neo-aorta and to the systemic–pulmonary shunt [4,5].
In our reported cases we created an end-to-end anastomosis for reconstruction of the neoaorta. An other technique of direct anastomosis was published by Brawn et al. [6].
Nevertheless an end-to-end anastomosis between two vessels is technically less complicated than either Brawn's technique or using a homograft gusset. Our suggested technique also shortens the circulatory arrest time, but requires extensive mobilisation of the vessels. On the other hand to create an end-to-end anastomosis the distal ductal–aortic junction was used. Although all macroscopically visible ductal tissue was excised, the probability exists that microscopic segments of ductal cells remain. However, at follow up no stricture was seen at or above the anastomosis site by echocardiography, but further observation is obviously necessary.
In our technique the tiny ascending aorta was ligated at its mid portion and a side-to-side anastomosis was created between the proximal remnant of it and the neo-aorta just above the valve level. With this approach the coronaries are perfused directly, not retrograde through the ascending aorta, therefore, the risk of coronary perfusion problem is minimal [7].
The systemic–pulmonary shunt in two cases was created without using a graft. Sizing a natural systemic–pulmonary shunt is more difficult than using a Gore-tex tube, but if successful it remains open without aspirin long term. In both cases a carefully constructed innominatepulmonary artery shunt led to satisfactory haemodynamics and oxygen saturation. This may lead to more varied management options in the future.
Overall we think that if the anatomy is favourable, repair of aortic atresia and hypoplastic left heart syndrome can be performed without using graft material.
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References
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Fyler D.C. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980;65:377-461.[Abstract]
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Noonan J.A., Nadas A.S. The hypoplastic left heart syndrome: an analysis of 101 cases. Pediatr Clin North Am 1958;5:1029-1056.[Medline]
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Norwood W.I., Lang P., Hansen D.D. Physiologic repair of aortic atresia–hypoplastic left heart syndrome. N Engl J Med 1983;308:23-26.[Medline]
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Meliones J.N., Snider A.R., Bove E.L., Rosenthal A., Rosen D.A. Longitudinal results after first stage palliation for hypoplastic left heart syndrome. Circulation 1990;82(Suppl IV):IV 151-156.
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Murdison K.A., Baffa J.M., Farrel P.B., Chang A.C., Barber O., Norwood W.I., Murphy J.D. Hypoplastic left heart syndrome. Outcome after initial reconstruction and before modified Fontan procedure. Circulation 1990;82(Suppl IV):IV199-207.
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Bu'Lock F.A., Stümper O., Jagtap R., Silove E.D., De Giovanni J.V., Wright J.G.C., Sethia B., Brawn W.J. Surgery for infants with a hypoplastic systemic ventricle and severe outflow obstruction: early results with a modified Norwood procedure. Br Heart J 1995;73:456-461.[Abstract/Free Full Text]
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Fraser C.D., Mee R.B.B. Modified Norwood procedure for hypoplastic left heart syndrome. Ann Thor Surg 1995;60:S546-S549.
Received September 28, 1999;
received in revised form November 1, 1999;
accepted November 24, 1999.
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Abstract
1. Introduction
