Ann Thorac Surg 1999;68:1850-1852
© 1999 The Society of Thoracic Surgeons
Case Reports
Common arterial trunk associated with double aortic arch
Christian Schreiber, MDa,
Victor T. Tsang, FRCSa,
Robert Yates, MRCPb,
Sachin Khambadkone, MRCPb,
Siew Yen Ho, FRCPathc,
Robert H. Anderson, MDc
a Department of Cardiothoracic Surgery, Great Ormond Street Hospital for Sick Children, NHS Trust and the Institute of Child Health, London, England, UK
b Department of Cardiology, Great Ormond Street Hospital for Sick Children, NHS Trust and the Institute of Child Health, London, England, UK
c Section of Pediatrics, Imperial College School of Medicine at the National Heart & Lung Institute, London, England, UK
Address reprint requests to Dr Schreiber, German Heart Center at the Technical University of Munich, Lazarettstrasse 36, 80636 Munich, Germany
e-mail: schreiber{at}dhm.mhn.de
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Abstract
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The combination of common arterial trunk associated with double aortic arch is very rare. We are aware of only four cases ever reported in English literature. We add two cases of this entity and comment on the morphological aspects, the clinical impact of the combined lesions, and their diagnostic and therapeutic implications.
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Introduction
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Malformations of the aortic arch may occur as isolated lesions, but in many cases they are associated with other cardiac or cardiovascular anomalies. The clinical presentation and surgical approach vary depending on the specific combination, with double aortic arch being the most florid example of the family of so-called vascular rings [1]. A common arterial trunk is the entity in which one arterial trunk directly supplies the coronary arteries and both systemic and pulmonary circulation [2]. It, too, can be associated with many and varied cardiac or cardiovascular malformations [3, 4]. We describe here two cases of common arterial trunk associated with double aortic arch. As far as we are aware, this combination has been previously encountered on only four occasions, each time as a solitary case (Table 1). We discuss the clinical impact of the combined lesions, and their diagnostic and therapeutic implications.
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Case reports
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Patient 1
The first patient presented with cyanosis after birth; the cyanosis was treated initially with a prostaglandin infusion. Echocardiography demonstrated a common arterial trunk with a large, unrestrictive ventricular septal defect. The defect was perimembranous and directly juxtaarterial. The truncal valve arose predominantly from the right ventricle. The pulmonary arteries had a confluent segment arising from the anterior and left-sided surface of the common arterial trunk, the origin of this confluent segment being adjacent also to the origin of the right coronary artery. Chromosomal analysis revealed 22q11 deletion. Further echocardiographic examination revealed the presence of a double aortic arch (Figs 1A and 1B). Surgery was undertaken at the 11th day of life, when the anticipated diagnosis was confirmed. The two arches were of almost comparable size (Fig 1C) and the truncal valve possessed four dysplastic cusps. The slightly narrowed segment of the left arch beyond the origin of the left common carotid artery was divided and oversewn. On bypass, the confluent pulmonary arterial segment was then detached from the arterial trunk, the remaining defect was patched, and the ventricular septal defect was closed using circulatory arrest. During rewarming, a homograft was used to reestablish the continuity between the right ventricle and the pulmonary arteries. The postoperative course was complicated by Klebsiella septicemia. Echocardiography at discharge showed good biventricular function, no residual defects, and mild truncal valvar insufficiency. The patient had no signs of respiratory or digestive problems.
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Fig 1. (A) Suprasternal short-axis echocardiographic image showing approximately equal sized right and left aortic arch. (L Arch = left aortic arch; R Arch = Right aortic arch; PT = pulmonary trunk.) (B) Echocardiographic image, subcostal view, demonstrating common arterial trunk dividing into right and left aortic arches. Note proximal origin of the main pulmonary artery. (CAT = common arterial trunk; PT = pulmonary trunk.) (C) Surgeon’s view of the common arterial trunk with the branching of the aortic arches and the pulmonary confluent.
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Patient 2
The second patient was in cardiac failure and the truncal valve was incompetent. At the time of presentation, in 1976, the decision was taken to undertake initial palliation, but banding of the pulmonary arterial segment through a left anterior lateral thoracotomy did not improve the clinical condition. The patient died a few days after surgery. Postmortem examination revealed a grossly dysplastic truncal valve, and a double aortic arch (Fig 2). The pulmonary band had been placed around a confluent segment feeding the right and left pulmonary arteries, producing a narrowing but not an obstruction.
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Fig 2. The specimen showing the double arch in combination with common arterial trunk and the proximal origin of the pulmonary confluence.
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Comment
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Recognition of the presence of a common arterial trunk gives no indication of the number and variety of associated cardiac and cardiovascular anomalies. For full diagnosis, complete sequential segmental analysis, including a catalogue of the anticipated associated anomalies, is essential. Anomalies of the aortic arches include the frequent right-sided aortic arch, interruption of the aortic arch, vascular rings due to variations in the origins of the brachiocephalic arteries, and the extremely rare double arch. Double aortic arch is usually associated with other cardiac malformations and very rarely with common arterial trunk, but can also present in isolation [1]. At the same time, there is a strong link between the presence of chromosome 22q11 deletions, Di George syndrome, and malformations of the ventricular outflow tracts, pointing to the importance of screening in the setting of either double arch or common trunk [5].
It is important to bear all these possible associations in mind when assessing the patient with common arterial trunk and associated malformations of the aortic arch. In the rare setting of double aortic arch, both arches can be visualized directly echocardiographically by performing standard suprasternal long-axis investigation and rotating the transducer. Subcostal views of the left ventricular outflow tract may demonstrate the aorta bifurcating into its two branches. Doppler color flow mapping helps demonstrate the origins of the brachiocephalic vessels [6]. The combination with common arterial trunk produced no particular problems during the surgical correction.
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Acknowledgments
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Robert H. Anderson and Siew Yen Ho are supported by the British Heart Foundation, together with the Joseph Levy Foundation.
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References
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Kocis K.C., Midgley F.M., Ruckman R.N. Aortic arch anomalies. Pediatr Cardiol 1997;18:127-132.[Medline]
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Anderson R.H., Thiene G. Categorization and description of hearts with a common arterial trunk. Eur J Cardiothorac Surg 1989;3:481-487.[Medline]
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Calder L., Van Praagh R., Van Praagh S., et al. Truncus arteriosus communis. Clinical, angiocardiographic, and pathologic findings in 100 patients. Am Heart J 1976;92:23-38.[Medline]
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Collett R.W., Edwards J.E. Persistent truncus arteriosus. Surg Clin North Am 1949;29:1245-1269.[Medline]
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Goldmuntz E., Clark B.J., Mitchell L.E., et al. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol 1998;32:492-498.[Abstract/Free Full Text]
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Snider AR, Serwer GA, Ritter SB. Abnormal vascular connections and structures. In: Snider AR, Serwer GA, Ritter SB, eds. Echocardiography in pediatric heart disease, 2nd ed. St. Louis: Mosby-Year Book, 1997:478–9.
Accepted for publication April 19, 1999.
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Abstract
Introduction
