Ann Thorac Surg 2006;82:2300-2302
© 2006 The Society of Thoracic Surgeons
Case Reports
Stage I Palliation for Interrupted "Right" Aortic Arch Associated With Mitral Atresia
James D. St. Louis, MD*
Department of Cardiothoracic Surgery, The Medical College of Georgia, Augusta, Georgia
Accepted for publication March 27, 2006.
* Address correspondence to Dr St. Louis, Department of Cardiothoracic Surgery, Medical College of Georgia, 1120 15th St., BA-4300, Augusta, GA 30912. (Email: jstlouis{at}mcg.edu).
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Abstract
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A 2.6 kg female with the diagnosis of type "B" interruption of the "right" aortic arch and mitral atresia underwent stage I palliation with translocation and anastomosis of the distal right carotid artery to the descending aorta. The distal main pulmonary artery was anastomosed to the under surface of the neoaortic arch. A modified Blalock-Taussig shunt was constructed between the left side innominate artery and the left pulmonary artery. The child awaits stage II palliation.
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Introduction
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Patients with complex univentricular cardiac anatomy and an interrupted aortic arch present a unique therapeutic challenge in attempting palliative surgical care. Interrupted aortic arch will generally present with a large posteriorly malaligned ventricular septal defect and occasionally mild to moderate stenosis of the left side structures. Mitral atresia occurs in less than 2% of patients with interrupted aortic arch and has an associated operative mortality of greater than 60% [1]. Initial repair requires establishment of aortic arch continuity in combination with stage I single ventricle palliation. Reconstruction of the aortic arch should include use of the native tissue to provide ample opportunity for growth while avoiding compression of vital structures. Use of the carotid artery to establish aortic continuity in a two-ventricle repair of interrupted aortic arch has been reported with good mid-term results and low neurologic complications [2, 3]. We believe that the use of a carotid artery in conjunction with a stage I single ventricle palliation has not been previously reported.
The patient being reported is a 2.6 kg female with a prior in utero diagnosis of hypoplastic left heart syndrome. The transthoracic echocardiogram demonstrated mitral atresia, severe hypoplasia of the left ventricle, and a hypoplastic ascending aorta measuring 3 mm. There was a moderate sized perimembranous ventricular septal defect. The main pulmonary artery measured 11 mm and the right-sided ductus arteriosus was in continuity with a right-sided descending aorta. The right aortic arch was not well visualized, but the morphology of the great vessels was consistent with an interrupted aortic arch. The situs on the child was ambiguous with dextrocardia. There were bilateral superior venae cavae. The left-sided inferior vena cava was interrupted with continuation to the left-sided superior vena cava.
Cardiac cauterization established the diagnosis of a type "B" interruption of a right-sided aortic arch. The first branch of the aortic arch was a left innominate artery. This was followed by the right carotid artery. Blood flow to the descending aorta was provided by the ductus arteriosus (Fig 1). There was antegrade flow across the aortic annulus.
The patient was taken to the operating room on day 8 of life. The arch anatomy was as described by catheterization. The ascending aorta was found to be 4 mm in diameter. The ductus arteriosus inserted onto the descending aorta at the origin of the right subclavian artery.
A 3.0 modified Blalock-Taussig shunt was fashioned between a left innominate artery and the left pulmonary artery. This was occluded during cardiopulmonary bypass and reopened at removal from bypass. Arterial cannulation was accomplished through the distal ascending aorta and the ductus arteriosus. Venous cannulation was performed through the left-sided right atrial appendage. Cardiopulmonary bypass was initiated and the patient was cooled to 18°C. The ductus arteriosus was occluded around the cannula and the main pulmonary artery was transected at its bifurcation. The pulmonary artery confluence was repaired with a piece of autologous pericardium. After sufficient cooling, the systemic circulation was arrested, the left innominate artery occluded, and the arterial cannulas were removed. The ductus arteriosus was completely resected. The right carotid artery was transected proximal to its bifurcation. The distal end of the carotid was turned down to the right and partially anastomosed to the proximal end of the descending aorta. The underneath surface of the neoaortic arch (ie, the right carotid artery) was opened from its distal end to its origin. The distal main pulmonary artery was then anastomosed to the inferior edge of the neoaortic arch in an end-to-side fashion (Fig 2). The arterial cannula was placed into the main pulmonary artery and flow was re-established. The patient was weaned from cardiopulmonary bypass with 5 ug/min/kg of dopamine and 0.5 ug/min/kg of milrinone. Postoperative cardiac catheterization accomplished at 3 months of age, which revealed a peak gradient of 10 mm Hg across the reconstructed arch with a measured right ventricular end diastolic pressure of 12 mm Hg. There was no branch pulmonary artery stenosis. There was mild narrowing of the origin of the modified Blalock-Taussig shunt. The patient now awaits bilateral bidirectional Glenn operation. Institutional approval was not required for this study.
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Fig 2. Illustration of the complete stage I palliation using the right carotid artery to establish aortic continuity.
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Comment
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Survival after stage I palliation for univentricular hearts associated with reconstruction of either hypoplastic or atretic aortic arch has improved significantly over the last decade. Institutions are now reporting in-hospital survival rates of over 75% [4]. Several factors have contributed to overall improved outcomes, including improved technical expertise, superior intensive care management, and better out-of-hospital follow-up. Despite these improvements, major associated cardiac anomalies associated with single ventricle palliative procedures continue to plague both short-term and long-term survival [5]. Kanter and colleagues [6] presented excellent midterm results with the application of the Norwood principle for infants without hypoplastic left heart syndrome. Two patients in his series underwent a Norwood type operation for type B interrupted aortic arch and subaortic obstruction. Both of these patients underwent primary repair of the arch interruption before enlargement of the hypoplastic aorta with a homograft patch. They eventually went on to have a biventricular repair with no reported complication related to the use of homograft material. Our patient presented with the unique anatomic combination of anomalies, which included a right aortic arch associated with a long interrupted segment. Primary repair of the aortic arch would have risked long term complications that have been associated with Mee’s modification of the Norwood operations [7]. The use of a prosthetic conduit as described by others [8] was not considered because of the ultimate need for replacement and possible midterm obstructive effects on single ventricle physiology.
In summary, this patient presented a constellation of defects that made conventional surgical techniques difficult. We used native arterial tissue to provide the best chance for growth and avoid long-term compression of vital structures. Short-term follow-up by catheterization revealed good growth of the right pulmonary artery with no hemodynamically significant arch stenosis. Long-term follow-up and adequate candidacy for a bilateral bidirectional Glenn procedure will provide validation for this unorthodox reconstruction.
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References
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- Jones RA, Quaegebeur JM, Kirklin JW, Blackstone EH, Daicoff G. Outcomes in patients with interrupted aortic arch and ventricular septal defect: a multiinstitutional study J Thorac Cardiovasc Surg 1994;107:1099-1113.[Abstract/Free Full Text]
- Hakimi M, Clapp SK, Walters HL, Lvons JM, Morrow WR. Arch growth after staged repair of interrupted aortic arch using carotid artery interposition Ann Thorac Surg 1997;64:503-507.[Abstract/Free Full Text]
- Tlaskal T, Hucin J, Hruda J, et al. Results of primary and two stage repair of interrupted aortic arch Eur J Cardiothorac Surg 1998;14:235-242.
- Ohye RG, Gomex CA, Goldberg CS, Graves HL, Davaney EJ, Bove EL. Tricuspid valve repair in hypoplastic left heart syndrome J Thorac Cardiovasc Surg 2004;127:465-472.[Abstract/Free Full Text]
- Gaynor JW, Mahle WT, Cohen MI, et al. Risk factors for mortality after the Norwood procedure Eur J Cardiothorac Surg 2002;22:82-89.[Abstract/Free Full Text]
- Kanter KR, Miller BE, Cuadrade AG, Vincent RN. Successful application of the Norwood procedure for infants without hypoplastic left heart syndrome Ann Thorac Surg 1995;59:301-304.[Abstract/Free Full Text]
- Fraser CD, Mee RB. Modified Norwood procedure for hypoplastic left heart syndrome Ann Thorac Surg 1995;60:S546-S549.[Medline]
- Turley K. Growth of the hypoplastic ascending aorta after radical palliation Ann Thorac Surg 1991;52:647-651.[Abstract]
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Abstract
Introduction
