Ann Thorac Surg 1998;66:264-267
© 1998 The Society of Thoracic Surgeons
Case Reports
One-stage repair of interrupted aortic arch, aortopulmonary window, and anomalous origin of right pulmonary artery with autologous tissues
Massimiliano Codispoti, MDa,
Pankaj S. Mankad, FRCS(C/Th)a
a Department of Cardiac Surgery, Royal Hospital for Sick Children, Edinburgh, Scotland, United Kingdom
Accepted for publication February 7, 1998.
Address reprint requests to Mr Mankad, Department of Cardiac Surgery, Royal Hospital for Sick Children, Edinburgh EH9 1LF, Scotland
e-mail: (pankaj.mankad{at}ed.ac.uk)
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Abstract
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Interruption of the aortic arch, distal aortopulmonary septal defect, and aortic origin of the right pulmonary artery with intact interventricular septum were associated findings in a 4-month-old infant who underwent successful one-stage repair without the use of any synthetic material, thus allowing for a potential for growth of both the pulmonary artery and the aorta. The baby made an uneventful recovery and remains asymptomatic 36 months after repair.
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Introduction
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The rare association of aortopulmonary window (APW), aortic arch interruption, and anomalous origin of the right pulmonary artery (RPA) has previously been recognized as a syndrome [1]. Nevertheless, recognition of all the features by echocardiography and angiography remains a difficult task [2]. Although sporadic reports of surgical treatment appear in the literature, early successful results are rarely achieved [3, 4]. Even when these are obtained, there generally is no potential for growth of either the pulmonary arteries or the aorta [5]. We report successful surgical treatment of this complex anomaly in the same setting by a combined sternotomy-thoracotomy approach using only the native tissues.
A 4-month-old infant weighing 3.4 kg was referred because of difficulty in feeding. On physical examination he was not tachypneic, he had a quiet precordium, and the heart sounds were normal, with a grade 3/6 systolic murmur best audible posteriorly. His electrocardiogram was normal, and chest radiography showed mild cardiomegaly with some increase in pulmonary venous markings. Echocardiography revealed interruption of the aortic arch and an aortopulmonary septal defect. Cardiac catheterization and angiography confirmed the diagnosis of a nonrestrictive proximal APW and aortic arch interruption between the left carotid and the left subclavian arteries (type B interrupted aortic arch by the grading system of Celoria and Patton [6]). During the following days the baby experienced several epileptic episodes, and a computed tomographic scan showed a focal discrete ischemic patch within the right-sided basal ganglia. Because of the risk of hemorrhage into an infarcted area during systemic anticoagulation, and taking into account the good general condition of the baby, operation was deferred for 1 month.
At 5 months of age the baby underwent operation for total repair through a median sternotomy. At operation, in addition to the above findings, an anomalous origin of the RPA from the right lateral aspect of the distal portion of the ascending aorta was observed (Fig 1). There was a 1.5-cm long segment of interruption between the left common carotid artery and the left subclavian artery. Cardiopulmonary bypass was established by inserting the arterial cannula on the right lateral wall of the ascending aorta, as distal to the APW as possible, and by single venous cannulation. During cooling to 16°C, extensive dissection of the aortic arch and the descending thoracic aorta was achieved. The left subclavian artery and the first pair of intercostal arteries were ligated and divided to avoid any tension on the aortic anastomosis. During 37 minutes of circulatory arrest, after as much ductal tissue had been resected as possible, an end-to-side anastomosis of the descending aorta to the undersurface of the aortic arch was performed with a 7/0 polypropylene suture. Low-flow bypass was resumed and during cardioplegic arrest the ascending aorta was incised longitudinally over the APW for 1.5 cm. Inspection of the internal anatomy confirmed the anomalous origin of the RPA from the ascending aorta. The internal diameters of the APW and of the RPA orifice measured 7 mm and 6 mm, respectively. Therefore, the incision was extended transversely in both directions, transecting the ascending aorta in such a way as to leave as much posterior aortic cuff as possible to form the posterior wall of the confluence between the right and the left pulmonary arteries (Fig 2). Anteriorly, the pulmonary artery was reconstructed by anastomosis of an autologus pericardial patch with a continuous 6/0 polypropylene suture (Fig 3). The two segments of the ascending aorta were directly anastomosed with a continuous 7/0 polypropylene stitch; the suture line was interrupted in two places to avoid a pursestring effect. Soon after reperfusion the patient reverted spontaneously to sinus rhythm and cardiopulmonary bypass was successfully discontinued at normothermia on 10 µg · kg-1 · min-1 of dopamine. Total bypass time was 2 hours 45 minutes, with a cross-clamp time of 46 minutes and a period of circulatory arrest of 37 minutes.
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Fig 2. The ascending aorta is divided, leaving its posterior wall to form the posterior half of the right pulmonary artery.
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After transfer to the intensive care unit, invasive monitoring of femoral arterial pressure was obtained (cannulation was not possible preoperatively), and it demonstrated a residual peak gradient of 30 mm Hg across the aortic anastomosis (right radial artery pressure = 70/35 mm Hg; femoral artery pressure = 40/20 mm Hg). Although the baby was peripherally warm, well perfused, and not developing metabolic acidosis, he remained anuric. After 4 hours the patient was brought back to the operating room for repair of residual stenosis at the aortic arch repair site. Via a left thoracotomy, with surface-induced moderate hypothermia (30°C), we occluded the aortic arch beyond the origin of the innominate artery and the descending thoracic aorta with vascular clamps. The lateral aspect of the anastomosis was taken down and an autologous pericardial patch was sewn in to enlarge the anastomotic site. The clamps were in place for 30 minutes. After repair, there was no residual gradient across the anastomosis by direct pressure measurement.
The baby made an uneventful recovery and was discharged home on the fifteenth postoperative day. An echocardiogram performed at the time of discharge revealed a satisfactory repair, with a residual peak Doppler gradient at the level of the RPA reconstruction of 15 mm Hg and a 12 mm Hg gradient at the site of the repair of the aortic interruption.
At 36 months of follow-up the patient is growing satisfactorily and remains free of symptoms. Repeat Doppler echocardiography confirmed the same findings described above.
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Comment
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Interrupted aortic arch, aortopulmonary septal defect, and aortic origin of the RPA is a rare anomaly, which poses challenging problems to both the cardiologist and the cardiac surgeon. Correct diagnosis is difficult to establish even with the aid of two-dimensional echocardiography, cardiac catheterization, and angiography [2], and a satisfactory repair can be regarded as such only when it results in an unobstructed left ventricular outflow tract, aortic arch, and RPA. Ideally, the growth potential of the aorta and the pulmonary artery should be maintained. The results of the one-stage repair of interrupted aortic arch and associated cardiac anomalies are relatively better than those of a two-stage approach in terms of both mortality and morbidity [5]. However, even when survival is achieved, the use of prosthetic material to reconstruct either the aorta or the pulmonary artery impairs the future growth potential of these structures [3, 7, 8].
In our patient, complete repair of interrupted aortic arch, APW, and anomalous origin of the RPA has been achieved without the use of any synthetic material while avoiding circumferential suture lines on all the anastomoses. The posterior wall of the ascending aorta was used to form the posterior portion of the confluence between the RPA and the main pulmonary artery, and a patch of autologous pericardium was used to constitute its anterior half, a technique previously described by Kitagawa and colleagues [9] in a patient with a distal aortopulmonary septal defect. It may not be necessary to shift the pulmonary arteries in front of the aorta, as suggested by Burke and Rosenfeld [4], to avoid compression of this structure by the aortic arch, even in the presence of an interrupted aortic arch. In fact, we believe that an extensive mobilization of the entire aortic arch and descending aorta, obtainable through a combined sternotomy-thoracotomy approach, allows a tension-free aortic anastomosis without increasing the risk of the operation. Since we operated on this patient, we have used the combined approach to perform repair in 3 more infants with interrupted aortic arch (with a gap between the two aortic stumps longer than 1.5 cm) and associated anomalies: the whole length of the descending thoracic aorta is first mobilized via a left thoracotomy, the wound is closed, the patient is turned in the supine position, and the repair is completed via the sternotomy approach. We had no incidence of residual stenosis in these patients. However, we appreciate that the alternative approach to mobilize as much descending aorta as possible from the midline sternotomy is a perfectly safe and feasible proposition, especially when the interrupted segment is short.
In summary, we report a case of type B interrupted aortic arch, aortopulmonary septal defect and anomalous origin of the RPA in which we performed successful one-stage repair avoiding the use of any synthetic material, thus allowing the potential for future growth.
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References
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- Berry T.E., Bharati S., Muster A.J., et al. Distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and hypoplasia of the aortic isthmus: a newly recognized syndrome. Am J Cardiol 1982;49:108-116.[Medline]
- Fisher E.A., Du Brow I.W., Eckner F.O., Hastreiter A.R. Aorticopulmonary septal defect and interrupted aortic arch: a diagnostic challenge. Am J Cardiol 1974;34:356-359.[Medline]
- Ding W.X., Su Z.K., Cao D.F., Jonas R.A. One-stage repair of the aortopulmonary septum and interrupted aortic arch. Ann Thorac Surg 1990;49:664-666.[Abstract]
- Burke R.P., Rosenfeld H.M. Primary repair of aortopulmonary septal defect, interrupted aortic arch, and anomalous origin of the right pulmonary artery. Ann Thorac Surg 1994;58:543-545.[Abstract]
- Menhahem S., Rahayoue A.U., Brawn W.J., Mee R.B. Interrupted aortic arch in infancy: a 10-year experience. Pediatr Cardiol 1992;13:214-221.[Medline]
- Celoria G.C., Patton R.B. Congenital absence of the aortic arch. Am J Cardiol 1959;58:407-412.
- Dietl C.A., Torres A.R. Repair of interrupted aortic arch with an augmented aortic anastomosis. Ann Thorac Surg 1993;56:142-148.[Abstract]
- Jonas R.A. Modified arch anastomosis for interrupted aortic arch. Ann Thorac Surg 1993;56:55-56.
- Kitagawa T., Katoh I., Taki H., et al. New operative method for distal aortopulmonary septal defect. Ann Thorac Surg 1991;51:680-682.[Abstract]
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Abstract
Introduction
