Ann Thorac Surg 1995;60:1400-1402
© 1995 The Society of Thoracic Surgeons
Case Reports
Aortic Root Replacement and Extraanatomic Bypass for Interrupted Aortic Arch in an Adult
Ben J. L. Burton, MA,
Panny Kallis, FRCS,
Chris Bishop, FRCS,
R. Howard Swanton, FRCP,
Charles W. Pattison, FRCS
Departments of Cardiology, Cardiothoracic Surgery, and Vascular Surgery, The Middlesex Hospital, London, England
Accepted for publication April 26, 1995.
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Abstract
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We describe an unusual case of interrupted aortic arch, aneurysmal ascending aorta, and aortic regurgitation in a 24-year-old man. He presented with general malaise, weakness of his legs, and hypertension. A single-stage operation was performed in which the aortic root was replaced with concomitant extraanatomic bypass of the interrupted segment of the aortic arch. He made a full recovery and has returned to work.
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Introduction
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Interruption of the aortic arch (IAA) is a rare anomaly [1], often associated with other complex cardiac lesions, and has a poor prognosis [2]. Isolated IAA is rare and thought at one time to be incompatible with life once the ductus arteriosus closed. However, when the ductus is obliterated in fetal life, a collateral circulation is already present at birth and survival is possible [3]. Nevertheless the management of patients who survive undiagnosed into adulthood poses a challenging problem to the surgeon. We report a particularly difficult patient who had IAA, aneurysmal aorta, and aortic regurgitation, who was treated successfully with a one-stage operation.
A 24-year-old man first presented with a 6-week history of general malaise and lethargy. He also described weakness of both legs on exertion, but no claudication. There was no past medical history of note. On examination he had a blood pressure of 200/80 mm Hg in both arms. Both femoral pulses were weak with marked radial-femoral delay. The heart was mildly enlarged, and he had an early diastolic murmur with a soft ejection systolic murmur in the aortic area. He also had a loud systolic bruit over the right subscapular area. An electrocardiogram confirmed left ventricular hypertrophy. A chest roentgenogram showed bilateral rib notching. An echocardiogram showed moderate aortic regurgitation with enlargement of the aortic root (6.5 cm in diameter). A provisional diagnosis of aortic coarctation was made, and he was treated with antihypertensive drugs. Magnetic resonance imaging suggested a coarctation of the aorta just distal to the left subclavian artery with a dilated aortic root (Fig 1
). Cardiac catheterization was performed via the right brachial artery, which showed an interrupted arch distal to the left subclavian artery. There was also a vast innominate artery, tortuous left subclavian artery, and a huge left internal mammary artery. There was also aneurysmal dilatation of the aortic root with moderate aortic regurgitation, a dilated left ventricle with good function, and normal coronary arteries.
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Fig 1. . Magnetic resonance imaging demonstrating complete interrupted aortic arch, dilated aortic root, and hypoplastic descending aorta.
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A single-stage operation was carried out through a midline sternotomy and a left paramedian extraperitoneal exposure of the abdominal aorta. There was a thin-walled aneurysm of the ascending aorta, which tapered down to a small caliber just below the takeoff of the innominate artery. After exposure of the right common femoral artery, cardiopulmonary bypass was established through a two-stage venous cannula with arterial return both to the base of the innominate artery and to the right common femoral artery. Arterial pressure was monitored throughout from the right radial and the left common femoral artery. Myocardial protection was achieved by infusion of 1 L of cold crystalloid cardioplegia (St. Thomas' Hospital solution) directly into the coronary ostia. Cold normal saline solution was applied topically to the heart, and the patient was cooled to 28°C. The aortic valve was tricuspid and was excised. A 27-mm St. Jude aortic valved conduit (St. Jude Medical, St. Paul, MN) was inserted into the aortic root with multiple interrupted runs of 2/0 Prolene (Ethicon, Somerville, NJ). The left anterior descending and circumflex coronary arteries, which had separate orifices, and the right coronary were both reimplanted in the standard fashion with 5/0 Prolene. The distal end of the graft was then anastomosed to the ascending aorta just below the cross-clamp using continuous 3/0 Prolene. A button was then taken out of the ascending aortic graft and an 18-mm Dacron conduit (St. Jude Medical Ltd, Coventry, UK) was sewn into the ascending aorta end to side and tunneled into the left hemithorax through the left hemidiaphragm. The abdominal aorta was exposed using a left paramedian incision and an extraperitoneal approach. The Dacron conduit was anastomosed end-to-side to the infrarenal abdominal aorta, using local segmented occlusion. There were no perioperative complications, and the postoperative bleeding was minimal. Prophylactic aprotinin was used, however, from the beginning to the end of the surgical procedure.
The patient made an uneventful postoperative recovery and was discharged receiving atenolol, frusemide, enalapril, and warfarin. He was readmitted 3 weeks after discharge with chest pain, but repeat cardiac catheterization showed normal coronary artery flow and a functioning Dacron conduit (Fig 2A, B). Eleven weeks postoperatively he was readmitted with a self-terminating supraventricular tachycardia and a potassium level of 3.3 mmol/L. Six months after operation he remains well and active and has returned to work as an actor.
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Comment
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The simultaneous occurrence of IAA with aortic regurgitation, left ventricular dilatation, and an aneurysmal aortic root necessitated surgical intervention to avoid further deterioration of cardiac function and to avoid the risk of aortic rupture or dissection. Orthodox teaching would be to manage this type of complex pathology in two stages: To deal with the interrupted segment of the arch first through a left thoracotomy and then replace the aortic root through a midline sternotomy. In view of the degree of left ventricular dilatation and aortic regurgitation we thought that the morbidity and mortality of the first stage would be extremely high, so this aggressive single-stage procedure was adopted to try to improve the prognosis.
Single-stage repair of interrupted arch complex in infants is now thought to be the treatment of choice [4], although similar studies of adult patients are unavailable because of the rarity of the condition. Wong and colleagues [5] reported a case of isolated IAA in an asymptomatic adult who refused surgical intervention and who received antihypertensive treatment only. Kanoh and co-workers [6] reported the successful surgical treatment of solitary IAA using an extraanatomic Dacron graft from the ascending aorta to the abdominal aorta. Ohuchi and associates [7] performed an aortic root replacement and an extraanatomic bypass graft, but their case was for coarctation instead of complete IAA. Ueyama and colleagues [8] performed an extraanatomic bypass between the ascending aorta and descending aorta in a patient with coarctation of the aorta and right aortic arch. We conclude that aortic root replacement with concomitant extraanatomic bypass for complicated IAA offers an excellent single-stage strategy for this rare condition in adults.
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Footnotes
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Address reprint requests to Mr Pattison, University College London Hospital Trusts, Department of Cardiothoracic Surgery, The Middlesex Hospital, Mortimer St, London W1N 8AA, England.
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References
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- Roberts WC, Morrow AG, Braunward E. Complete interruption of aortic arch. Circulation 1962;26:39–59.[Abstract/Free Full Text]
- Collins-Nakai RL, Parisi-Buckley L, Fyler D, Castaneda AR. Interrupted aortic arch in infancy. J Pediatr 1976;88:959–62.[Medline]
- Dische MR, Tsai M, Baltaxe HA. Solitary interruption of the arch of the aorta. Clinicopathological review of eight cases. Am J Cardiol 1975;35:271–7.[Medline]
- Yasui H, Kado H, Yonenaga K, et al. Revised technique of cardiopulmonary bypass in one-stage repair of interrupted aortic arch complex. Ann Thorac Surg 1993;55:1166–71.[Abstract]
- Wong CK, Cheng CH, Lau CP, Leung WH, Chan FL. Inter-rupted aortic arch in an asymptomatic adult. Chest 1989;96:678–9.[Abstract/Free Full Text]
- Kanoh T, Yamanaka O, Kobayashi S, et al. An operative case of solitary interruption of the aortic arch in adult. Kokyu To Junkan 1991;39:367–72.[Medline]
- Ohuchi H, Kawazoe K, Kosakai Y, Kitoh Y, Kawashima Y. One stage repair for coarctation of the aorta and annuloaortic ectasia with severe aortic regurgitation in a patient with Turner syndrome. Nippon Kyobu Geka Gakkai Zasshi 1992;40:2247–51.[Medline]
- Ueyama K, Urayama H, Takemura H, Tsuchida K, Kato A, Watanabe Y. Coarctation of aorta with right aortic arch and anomalous left subclavian artery. Nippon Kyobu Geka Gakkai Zasshi 1992;40:1299–303.[Medline]
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Abstract
Introduction
