Eur J Cardiothorac Surg 2002;22:457-459
© 2002 Elsevier Science NL
One-stage repair of aortic isthmus atresia, aortic arch hypoplasia and ascending aorta aneurysm via midline sternotomy and normothermic perfusion
O. Baron*,
J.P. Rémadi,
M. Lefevre,
D. Duveau
Department of Cardiovascular Surgery, Hôpital Laënnec, 44093 Nantes, France
Received 25 October 2001;
received in revised form 24 April 2002;
accepted 7 June 2002.
* Corresponding author. Tel.: +33-2-4016-5090; fax: +33-2-4016-5402
e-mail: olivier.baron{at}chu-nantes.fr
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Abstract
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One-stage repair of aortic isthmus atresia, aortic arch hypoplasia and ascending aorta aneurysm was performed via a standard midline sternotomy. Ascending aortoplasty was performed during a 19-min cardiac arrest. With the heart beating and continuous normothermic perfusion of the upper and lower part of the body, the aortic arch was then enlarged using the Amato technique and the harvested ascending aorta wall as autologous patch. A classical Crafoord procedure then restored continuity between the aortic arch and the descending thoracic aorta.
Key Words: Aortic atresia • Ascending aorta aneurysm • Normothermic cardiopulmonary bypass
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1. Introduction
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The combination of aneurysm of the ascending aorta and atresia of the aortic isthmus and hypoplasia of the aortic arch is rare. This case is reported in order to describe and discuss the strategy used to achieve complete correction by midline sternotomy under strict normothermia and the techniques used to enlarge the proximal and distal aortic arch.
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2. Material and method
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The diagnosis of aortic coarctation was suspected in a totally asymptomatic 6-year-old boy. Echocardiography revealed an aneurysm of the ascending aorta (diameter: 34 mm) with hypoplasia of the aortic arch (diameter: 7 mm). There were no signs of regurgitation or stenosis of the bicuspid aortic valve. The left ventricular wall was thickened (9–10 mm). Magnetic resonance imaging confirmed the three associated lesions (Fig. 1)
: aneurysm of the aortic arch, hypoplasia of the aortic arch and atresia of the aortic isthmus.
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Fig. 1. Magnetic resonance image showing the three associated lesions: atresia of the aortic isthmus, hypoplasia of the aortic arch, aneurysm of the ascending aorta.
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Repair was performed via a standard midline sternotomy. The first step consisted of wide dissection of the aortic arch and neck vessels. The ductus arteriosus was divided and the aortic isthmus was also widely dissected. Cardiopulmonary bypass was established with an arterial cannula in the right femoral artery and another in the brachiocephalic trunk. A single venous cannula was placed in the right atrium and a vent in the left atrium. Temperature was maintained at 37 °C throughout the surgical procedure.
The heart was stopped with total blood cardioplegia containing potassium chloride and magnesium sulphate. During ischaemic cardiac arrest, a longitudinal resection-plasty of the ascending aorta was performed reducing the ascending aorta diameter from 37 to 25 mm. The resected aortic wall was kept in saline, but a sample was also sent to the pathology department for examination, which did not show any systemic disease, especially no Marfan's syndrome. After 19 min of cardiac arrest, the heart was reperfused. By clamping the ascending aorta, the neck vessels and descending aorta, the aortic arch was isolated for repair while the upper and lower parts of the body were perfused under normothermia (120 ml/kg per min). A specific blood-line from the oxygenator to the ascending aorta allowed a specific normothermic myocardial perfusion (7–10 ml/kg per min). The pressure in the ascending aorta was not measured during coronary arteries perfusion and the time to reconstruct the aortic arch was 70 min.
The aortic isthmus was resected, confirming obstruction of the aortic isthmus. Surgical repair is depicted in Fig. 2
. The distal arch was then enlarged using the Amato technique. Rapidly, a longitudinal incision was made in the superior aspect of the arch between the carotid and subclavian arteries. A suture was started in the midpoint of the posterior lip of the incision and was carried upward, approximating the anterior and posterior walls of the left common carotid artery and left subclavian artery. Completion of the suture line anteriorly joined the proximal portions of the left carotid and subclavian arteries and enlarged the diameter of the hypoplastic arch. The autologous patch from the ascending aorta wall was tailored and secured in the concavity to enlarge the proximal arch. Finally, continuity between the descending thoracic aorta and the reconstructed aortic arch was restored. The child was extubated 2 h later and discharged on the seventh postoperative day following angiography.
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3. Discussion
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Aneurysms of the ascending aorta are rare in children. The essential aetiologies are mycotic, congenital dystrophies of elastic tissue, especially Marfan's disease, and obstructive syndromes of the aortic arch. The surgical treatment of aneurysms localized to the ascending aorta consists of either replacing the ascending aorta by a vascular prosthesis or homograft [1], or more conservative resection-longitudinal plasty [2]. In view of this patient's young age, the absence of arterial wall dystrophy, the integrity of the aortic sinus and the absence of functional lesions of the aortic valve, we decided to perform resection-plasty which presents the major advantage of not introducing any animal or synthetic foreign material.
A non-patent fibrous tract was observed between the aortic arch and descending thoracic aorta, corresponding to atresia of the aortic isthmus and not interruption of the aortic arch characterized by the absence of any anatomical continuity between the aortic arch and the descending thoracic aorta.
Deep hypothermia circulatory arrest has been largely used during aortic arch surgery, especially in children. However, comparison of two cohorts of children operated on for the same disease, with or without circulatory arrest, showed a harmful effect of circulatory arrest on short-term cerebral activity and was associated with delayed development and neurological sequelae 1 year after surgery [3]. In order to avoid this type of circulatory arrest, some authors perform selective cerebral perfusion during aortic arch repair in neonates [4]. Although Tchervenkov et al. [4] consider anterograde perfusion of the brachiocephalic trunk to be sufficient to ensure perfusion of the lower half of the body via collateral vessels, the same authors nevertheless consider it advisable to perfuse under deep hypothermia (18 °C). Comparison of children operated under circulatory arrest or by selective perfusion of the upper half of the body with a third group operated by perfusing the upper and lower halves of the body, based on enzyme assays, also clearly demonstrated the marked benefit of maintenance of whole-body perfusion [5]. We have therefore adopted this perfusion strategy allowing whole-body perfusion during repair of the aortic arch, as the heart is stopped for only 19 min during ascending aortoplasty. The absence of prolonged organ ischaemia allowed strict normothermic perfusion thereby avoiding the harmful effects of hypothermia.
The surgical principles for repair of the aortic isthmus and aortic arch were: (1) wide dissection of the aortic arch, supra-aortic vessels, aortic isthmus and descending thoracic aorta, at least its proximal part; (2) wide resection of the atresic and ductus zone; (3) large end-to-end anastomosis between the concavity of the aortic arch and the thoracic aorta. As the hypoplasia involved all of the aortic arch and as the tissues are less flexible at the age of 6 years than during the neonatal period, various techniques were used to enlarge the aortic arch. Excessive traction between the descending thoracic aorta and proximal arch could also induce bronchial compression and increase the risk of recurrent coarctation [6]. Some authors have proposed using the left subclavian artery as patch graft to enlarge the distal aortic arch [7]. The main advantage of the Amato plasty performed in our case is to preserve the left subclavian artery while allowing very satisfactory enlargement of the distal arch [8]. A patch graft enlargement plasty was performed in the proximal arch. This technique was initially proposed by Tchervenkov et al. [9] using pulmonary homografts as enlargement patches. However, recoarctations were still observed after this technique, probably because of immunological reasons, which is why Serraf et al. used an arterial autologous patch graft from the anterior surface of the pulmonary artery to perform this enlargement. In their series of 22 patients, the recoarctation rate was zero and growth of the aortic arch was harmonious, confirming satisfactory growth of autologous pulmonary tissue in a systemic position [10]. As our young patient did not present any systemic disease or any histological abnormalities of the aortic wall and as he presented a bicuspid aortic valve that could possibly require a Ross procedure at a later date, we decided to preserve the integrity of the pulmonary artery and used a patch graft raised from the wall of the ascending aorta.
Overall, this case illustrates an original perfusion strategy allowing repair of the aortic arch under strict normothermia, but also a reconstruction technique ensuring optimal preservation of the anatomical integrity without sacrificing any supra-aortic vessels and using a large autologous arterial patch graft.
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Footnotes
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Presented at the joint 15th Annual Meeting of the European Association for Cardio-thoracic Surgery and the 9th Annual Meeting of the European Society of Thoracic Surgeons, Lisbon, Portugal, September 16–19, 2001.
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References
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- Cooley D.A., Taylor F.M. Aneurysm of thoracic aorta in an infant treated by resection and arterial homograft. J Pediatr 1956;49:185-190.[Medline]
- Michaud P., Chassignole J., Termet J., Du Gres B., Teneriello F. Aneurysm of the ascending aorta in 23-month-old child treated successfully by resection-plasty. Lyon Chir 1970;66:129-132.[Medline]
- Bellinger D.C., Jonas R.A., Rappaport L.A., Wypij D., Wernovsky G., Kuban K.C., Barnes P.D., Holmes G.L., Hickey P.R., Strand R.D., Walsh A.Z., Helmers S.L., Constantinou J.E., Carrazana E.J., Mayer J.E., Hanley F.L., Castaneda A.R., Ware J.H., Newburger J.W. Developmental and neurologic status of children after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. N Engl J Med 1995;332:549-555.[Abstract/Free Full Text]
- Tchervenkov C.I., Korkola S.J., Shum-Tim D. Surgical technique to avoid circulatory arrest and direct arch vessel cannulation during neonatal aortic arch reconstruction. Eur J Cardiothorac Surg 2001;19:708-710.[Abstract/Free Full Text]
- Uemura H., Yagihara T., Kawahira Y., Yoshikawa Y., Kitamura S. Continuous systemic perfusion improves outcome in one stage repair of obstructed aortic arch and associated cardiac malformation. Eur J Cardiothorac Surg 2001;230:603-608.
- Karl T.R., Sano S., Brawn W., Mee R.B.B. Repair of hypoplastic or interrupted aortic arch via sternotomy. J Thorac Cardiovasc Surg 1992;104:688-695.[Abstract]
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- Amato J.J., Rheinlander H.F., Cleveland R.J. A method of enlargement the distal transverse arch in infants with hypoplasia and coarctation of the aorta. Ann Thorac Surg 1977;23:261-263.[Abstract]
- Tchervenkov C.T., Tahta S.A., Jutras L., Béland M.J. Single stage repair of aortic arch obstruction and associated intracardiac defects with pulmonary homograft patch aortoplasty. J Thorac Cardiovasc Surg 1998;116:897-904.[Abstract/Free Full Text]
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Abstract
1. Introduction
