Ann Thorac Surg 2006;82:1884-1886
© 2006 The Society of Thoracic Surgeons
Case Reports
Truncus Arteriosus With Interrupted Aortic Arch: Successful Repair Using Modified Cardiopulmonary Bypass and Surgical Techniques
Qiang Chen, MD, PhD,
Paul Modi, FRCS,
Massimo Caputo, MD,
Ash Pawade, FRCS*
Department of Cardiac Surgery, Bristol Royal Hospital for Children, Bristol, United Kingdom
Accepted for publication March 27, 2006.
* Address correspondence to Dr Pawade, Department of Cardiac Surgery, Bristol Royal Hospital for Children, Bristol, BS2 8HW United Kingdom. (Email: qchen{at}doctors.org.uk).
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Abstract
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A neonate with truncus arteriosus (type 1) and interrupted aortic arch (type A) associated with an excessively large gap between interrupted aortic segments underwent successful reconstruction of the aortic arch with a partial subclavian flap aortoplasty plus bovine jugular patch (Contegra 200, Medtronic, Inc, Minneapolis, MN) using continuous selective low-flow cardiopulmonary bypass without circulatory arrest. The ventricular septal defect was closed with a Dacron (IMPRA, Inc, Tempe, AZ) patch, and a Contegra prosthesis was used to re-establish right ventricle to pulmonary artery continuity. At an 18-month follow-up, the patient was free of symptoms with normal development. Echocardiography revealed a widely patent aortic arch and an unobstructed right ventricle to pulmonary artery connection.
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Introduction
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The conventional technique for one-stage repair of truncus arteriosus (TA) with interrupted aortic arch (IAA) is to reconstruct the aortic arch with direct anastomosis between the aortic arch and descending aorta under circulatory arrest, to close the ventricular septal defect, and to establish right ventricle to pulmonary artery (PA) continuity using a valved conduit or patch [1–10]. Recent reports have suggested direct anastomosis with patch augmentation to avoid the reintervention for aortic arch repair [7–10]. However, when there is an excessive long distance between the aortic arch and descending aorta, direct anastomosis may place undue tension on the arch and result in compression of other mediastinal structures. We present a successful reconstruction of IAA associated with an excessive large gap between two aortic segments using a combination of a subclavian flap and bovine jugular patch without circulatory arrest. The TA was corrected simultaneously using the conventional method.
A 3.1 kg female neonate presented with severe cardiac failure and gross metabolic acidosis on the first day of life. Transthoracic echocardiography demonstrated type I TA, type A IAA, a large patent ductus arteriosus and a muscular outlet ventricular septal defect. A prostaglandin infusion was commenced and on day 4 of life the primary repair was performed.
Median sternotomy revealed a considerably enlarged heart. The ascending aorta was of a normal size, but stopped shortly after the left subclavian artery, which was also normal in size. Both branch PAs were of good size and there was a large patent ductus arteriosus. The aortic arch, innominate artery, left common carotid artery, left subclavian artery, and the descending aorta were extensively dissected out. Cardiopulmonary bypass was established between bi-caval venous cannulas (Medtronic, Inc, Minneapolis, MN, single stage venous cannula, 67312 and 67314) and two 10-French straight arterial cannulae (Medtronic, Inc, Minneapolis, MN, DLP elongated one piece arterial cannula, 77010) placed separately into the aortic arch and descending aorta through the patent ductus arteriosus from the truncus. The branch PAs were snared and cardiopulmonary bypass was commenced. Nasopharyngeal temperature was dropped to 22°C. After cross clamping the aorta, myocardial protection was established with 25 mL/kg of cold blood cardioplegia infused into the aortic root supplemented by topical myocardial cooling. The right atrium was opened and the left ventricle was vented through the patent foramen ovale. The PA was detached from the aorta and the opening in the left side of the aorta was closed with a patch derived from a Contegra prosthesis (Metronic Contegra 200) using a continuous 7-0 polypropylene suture. The ventricular septal defect was exposed through a vertical right ventriculotomy and closed with a Dacron patch using interrupted pledgeted 6-0 polypropylene sutures.
Once the intracardiac repair was completed, the descending aortic cannula was removed and the ascending aortic cannula was redirected into the innominate artery to provide continuous perfusion to the brain. The cardiopulmonary bypass flow rate was dropped from 50 mL/kg/min to 15 mL/kg/min. The snares around the head and neck vessels were tightened. The patent ductus arteriosus was ligated, and a side biting clamp was applied to the descending aorta as far down as possible. The patent ductus arteriosus was then divided. The proximal descending thoracic aorta was trimmed back to remove all ductal tissue. There was a long segment of ductal tissue extending into the descending aorta. Its excision left a substantial gap between the two aortic segments. A direct anastomosis, under the circumstances, would have placed undue tension on the anastomosis, and caused compression of other mediastinal structures. Separating the two segments entirely with an artificial interposition graft would have necessitated further surgery. Therefore it was decided to reconstruct the arch by creating a tube using a combination of the native left subclavian artery flap and a prosthetic patch. The aortic arch was filleted open. The left subclavian artery was transected distally and its proximal part reflected down and sutured to the lateral lip of the descending aorta using a continuous 7-0 polypropylene suture. The entire medial aspect of the descending aorta was patched with a Contegra patch using a continuous 7-0 polypropylene suture (Fig 1).
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Fig 1. The interrupted aortic arch was repaired using a left subclavian flap along with a bovine pericardial patch in a patient with truncus arteriosus and interrupted aortic arch.
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The air was removed from the heart and the aorta was unclamped. The aortic cannula was redirected into the ascending aorta and the patient was rewarmed. A 12-mm Contegra prosthesis was anastomosed between the right ventriculotomy and PA bifurcation, while the empty beating heart was allowed to recover. After full systemic rewarming, cardiopulmonary bypass was discontinued with a systemic pressure of 50 mm Hg, a right atrial pressure of 9 mm Hg, and a left atrial pressure of 5 mm Hg. The PA pressure was half systemic. After modified ultrafiltration, the cannulae were removed and excess heparin was reversed with protamine sulphate. Good hemostasis was obtained and primary sternal closure was achieved without difficulty. The patient was transferred to the pediatric intensive care unit in stable condition with the use of nitric oxide and 5 mcg/kg/min of dopamine. Her postoperative course was essentially uneventful. The duration of ventilation was 3 days, the pediatric intensive care unit stay was 6 days, and the total hospital stay was 12 days. The postoperative echocardiogram showed a small pericardial effusion, good bi-ventricular function, peak velocity of right ventricle-PA conduit (1.2 m/sec), mild truncal valve regurgitation, no residual ventricular septal defect, and a widely patent aortic arch and descending aorta.
At the 18-month follow-up, the child remained free of symptoms with normal development and no neurologic deficiency. Echocardiography revealed good bi-ventricular function, a widely patent aortic arch, mild truncal valve regurgitation, and both peak velocities of right ventricle-PA conduit and aortic arch descending aorta less than 1.5 m/sec.
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Comment
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Truncus arteriosus with IAA is a rare and difficult condition to treat. One-stage repair in neonates has been possible using an end-to-side anastomosis technique for aortic arch repair combined with repair of TA using standard techniques [1–10]. It has been suggested that direct anastomosis with a patch augmentation technique for aortic arch reconstruction tended to provide the most durable repair [7–10]. However, in the presence of excessively large ductal tissue, its excision leaves a long gap between interrupted aortic segments. A direct anastomosis, under the circumstances, might potentially result in tension on the anastomosis and compression of the bronchovascular structures. A high incidence of repeat operations was reported because of obstruction or compression of the aortic arch, bronchus, and PAs [3]. In one of the largest series from a single institution reported by Sano and associates [3], 43% of patients required repeat operations for relief of obstruction of the arch and compression of the bronchus and PAs. In a recent multi-institutional retrospective cohort study of 50 neonates undergoing surgical interventions for TA + IAA, 7 of 22 survivors required a reintervention for post-IAA repair anastomotic obstruction [9].
However, in our case the distance between the two aortic ends was excessive and a direct anastomosis would have caused the problems previously stated. In addition, the presence of large branch PAs and the conduit after repair of the TA would have crowded the retro-aortic space even further. Techniques such as the Lecompte maneuver have been described to circumvent this problem [2, 11, 12], albeit with some controversies [13, 14]. In the present case, the subclavian flap was turned down to create the lateral portion of the descending aorta and the medial aspect of the descending aorta was formed by a bovine graft.
Autologous subclavian arterial flap has the advantage that it grows with the aorta. Augmentation of the aortic tract with bovine graft is especially useful when the aortic arch is hypoplastic, which is not uncommon in this type of malformation. In addition, this technique has the advantage of allowing the complete resection of ductal tissue while simultaneously avoiding any tension on the anastomosis or compression of the PA and bronchus.
Traditionally, deep hypothermic circulatory arrest has been used during the repair of the aortic arch. It has been recognized that periods of circulatory arrest exceeding 45 to 60 minutes are associated with significant risk of neurologic injury [15, 16]. In the current case, we used isolated low-flow perfusion of the head with a perfusate temperature of 22°C, as previously described. This technique not only provided adequate exposure and time for arch reconstruction, but also achieved the potential benefits from cerebral protection [15, 16].
In conclusion, we present a case of successful primary repair in a neonate with TA + IAA associated with an excessive large gap between interrupted aortic segments. The aortic arch repair was performed by creating a tube with a left subclavian flap augmented with a heterograft patch extending onto the inferior aortic arch under hypothermic isolated low-flow cerebral perfusion without circulatory arrest. The short-term outcome has been satisfactory; however, long-term follow-up is needed to assess the growth of the aortic arch and the descending aorta.
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References
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Abstract
Introduction
