Ann Thorac Surg 1996;62:887-888
© 1996 The Society of Thoracic Surgeons
Case Report
Type B Aortic Dissection Involving an Isolated Right-Sided Aortic Arch
Paolo Masiello, MD,
Generoso Mastrogiovanni, MD,
Giuseppe Santoro, MD,
Franco Triumbari, MD,
Giuliano Naimoli, MD,
Giuseppe Di Benedetto, MD
Divisions of Cardiac Surgery and Anesthesiology, Hospital "S. Giovanni di Dio e Ruggi D' Aragona," Salerno, Italy
Accepted for publication April 1, 1996.
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Abstract
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We report a case of a 48-year-old man in whom type B aortic dissection in the right aortic arch and right descending aorta was diagnosed by transesophageal echocardiography and computed tomographic scan. Angiography was necessary to define the anatomy of the branching vessels. The patient was successfully treated by interposition of a Vascutek 24-mm Dacron woven tube with a right posterolateral thoracotomy approach. Circulatory arrest in profound hypothermia and cerebral retroperfusion were used.
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Introduction
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Isolated right aortic arch is an uncommon congenital cardiovascular malformation in which the aortic arch overrides the right main bronchus and descends on the right side of the thoracic spine [1]. Most cases are diagnosed at pediatric age because of airway compression due to abnormal branching pattern of the emerging vessels (ie, aberrant left subclavian or left innominate artery and abnormal bilateral ductus) [2]. Conversely, it is unusually and occasionally diagnosed in adulthood.
In this report we describe an exceedingly rare case in which an isolated right-sided aortic arch was diagnosed at 48 years of age because of a type B aortic dissection.
A 48-year-old man with history of type II diabetes mellitus and moderate systemic hypertension was referred to our unit because of prolonged acute retrosternal pain radiating to the back. At the clinical examination no significant finding of cardiovascular disease was found. Systemic arterial pressure was 140/80 mm Hg and normal peripheral pulses were present. The electrocardiogram showed anomalies of the T wave at the precordial anterior leads. Chest roentgenogram revealed a normal-sized heart and a right-sided convex-shaped shadow (Fig 1
). The transesophageal echocardiography showed a type B aortic dissection and a right-sided aortic arch. A computed tomographic scan confirmed the diagnosis. We decided to catheterize the patient to assess the exact anatomy of the aorta and its branching vessels. The angiography showed a right-sided aortic arch with a descending aorta located on the right side in the thorax and resuming its normal position below the diaphragm. The maximum diameter of the aorta was 8 cm on its descending part. The intimal flap was located on the inferior side of the arch, just below the last neck vessel, and terminated 2 cm before the diaphragm (Fig 2). The brachiocephalic vessels arose separately from the arch; first, the left common carotid artery, then the right common carotid artery, the right subclavian artery, and the left subclavian artery (from Kommerel's diverticulum). Coronary angiograms showed normal coronary arteries and no evidence of atherosclerotic disease.
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Fig 2. . Aortogram in 30-degree anterior oblique view shows the intimal flap (F) and the left carotid artery (LCA) as first branching vessel.
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Surgical intervention was planned. A right posterolateral thoracotomy was made to expose adequately the aortic arch and the descending aorta (Fig 3). We decided to perform circulatory arrest because of the site of the dissection. The patient was put on right femorofemoral cardiopulmonary bypass and the superior vena cava was cannulated to perform cerebral retroperfusion at the time of the circulatory arrest. After cooling the patient to 18°C the perfusion was stopped and a Vascutek (Vascutek Ltd, Refrewshire, Scotland) 24-mm Dacron woven graft was inserted between the left subclavian artery, including the inferior part of the arch, and the descending aorta.
The postoperative course was uneventful and the patient was discharged home on the tenth postoperative day.
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Comment
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Right-sided aortic arch occurs in approximately 0.1% of the population. It can be classified in two major anatomic types, depending on the branching of the great vessels [3]. In the first type, the less common, so called mirror image, three great vessels arise from the arch in the following order: left innominate artery, right common carotid artery, and right subclavian artery. It is frequently associated with major cardiac anomalies, especially tetralogy of Fallot, truncus arteriosus, and tricuspid atresia [3]. The most common type, as in our patient, shows four great vessels arising from the arch in the following order: left common carotid artery, right common carotid artery, right subclavian artery, and left subclavian artery, the last arising from Kommerel's diverticulum. This type is less frequently associated to major cardiac anomalies (5% to 10%) [1, 3]. In contrast, tracheoesophageal compression may occur because the ductus arteriosus usually connects the left pulmonary artery to the left subclavian artery or to Kommerel's diverticulum, forming a vascular ring. However, most patients are asymptomatic and symptomatology in adulthood is rare [4].
Aortic dissection does not appear to have increased frequency in the right aortic arch, however, remaining a very rare case. To the best of our knowledge 3 patients have been reported in the literature [5–7], all of whom were treated surgically, 2 of them successfully. Circulatory arrest technique has been successfully used in correcting a descending aortic dissection with a double aortic arch [8]. In our patient we performed circulatory arrest because the position of the aneurysm precluded the application of the cross-clamp. Cerebral protection was obtained using retroperfusion of the cerebral vessels through the superior vena cava.
In conclusion, aortic dissection in the right aortic arch and right descending aorta is an extremely rare case that can be suspected based on the chest roentgenogram and easily diagnosed by transesophageal echocardiography and computed tomographic scan. Angiography is necessary to assess the exact anatomy of the lesion and of the great vessels and to plan the surgical intervention.
Right posterolateral thoracotomy is adequate to expose the descending aorta and provides a good operative field. When necessary, circulatory arrest is easy to perform and retroperfusion, made technically feasible by the right approach, can be used to provide cerebral protection throughout the procedure.
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Footnotes
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Address reprints requests to Dr Masiello, Piazza V. Veneto 39, 84123 Salerno, Italy.
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References
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- Ruckman RN. Anomalies of the aortic arch complex. In: Adams FH, Emmanouilides GC, Riemenschneider TA, eds. Moss' heart disease in infants and adolescents, 5th ed. Baltimore: Williams & Wilkins, 1994.
- Gidding S, Beekman R, Leibowitz E, et al. Airway compression by a right aortic arch in the absence of a vascular ring. Chest 1984;85:703–6.[Abstract/Free Full Text]
- Shuford WH, Sybers RG. The aortic arch and its malformations. In: Shuford WH, Sybers RG, eds. Right aortic arch. Springfield: Charles C. Thomas, 1974:52–68.
- Drucker MH, Symbas PN. Right aortic arch with aberrant left subclavian artery: symptomatic in adulthood. Am J Surg 1980;139:432–5.[Medline]
- Roan P, Parish S, Buja LM, et al. Dissecting aortic aneurysm involving a right-sided aortic arch. Am J Cardiol 1979;44:381–4.[Medline]
- Floten HS, Rose DM, Cunningham JN Jr. Surgical therapy of a dissecting aortic aneurysm involving a right sided aortic arch. J Am Coll Cardiol 1984;4:1058–61.[Abstract]
- Otheki H, Itoh T, Watanabe Y, Ueno T, Minato N, Natsuaki M. A dissecting aneurysm involving a right-sided arch, right descending aorta and mitral regurgitation. Thorac Cardiovasc Surg 1987;35:235–7.[Medline]
- Midulla P, Dapunt OE, Sadeghi AM, Quintana CS, Griepp RB. Aortic dissection involving a double aortic arch with a right descending aorta. Ann Thorac Surg 1994;58:874–5.[Abstract]
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S. Kuki, K. Taniguchi, S. Miyagawa, and H. Takano
Dissecting thoracoabdominal aortic aneurysm associated with an isolated right-sided aortic arch
Eur. J. Cardiothorac. Surg.,
May 1, 2000;
17(5):
614 - 616.
[Abstract]
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