Arteria lusoria: Developmental anatomy, clinical, radiological and surgical aspects

doi:10.1016/j.ancard.2009.07.008

Annales de Cardiologie et d'Angéiologie

Volume 59, Issue 3, June 2010, Pages 147-154

https://doi.org/10.1016/j.ancard.2009.07.008 Get rights and content

Abstract

The left aortic arch with an aberrant right subclavian artery, or arteria lusoria, is the most common aortic arch anomaly, occuring in 0.5–2.5% of individuals. Four vessels arise sequentially from the aortic arch: the right common carotid artery, the left common carotid artery, the left subclavian artery and the aberrant right subclavian artery, which crosses upwards and to the right in the posterior mediastinum. It results from a disruption in the complex remodelling of the paired branchial arches, typically of the right dorsal aorta distal to the sixth cervical intersegmental artery. The diagnosis and differentiation of arch anomalies is based on findings at chest radiography in association with those at esophagography. It is usually asymptomatic. When symptomatic, it produces dysphagia lusoria or dyspnea and chronic coughing. Treatment is indicated for symptomatic relief of dysphagia lusoria and for prevention of complications due to aneurysmal dilatation.

Résumé

L’arc aortique gauche avec artère sous-clavière droite aberrante ou arteria lusoria, est l’anomalie de l’arc aortique la plus fréquente, avec une prévalence de 0,5–2,5 %. Quatre vaisseaux proviennent séquentiellement de l’arc aortique : l’artère carotide commune droite, l’artère carotide commune gauche, l’artère sous-clavière gauche et l’artère sous-clavière droite aberrante, qui croise vers le haut et la droite dans le médiastin postérieur. Elle résulte d’une disruption dans le remodelage complexe des arcs branchiaux, typiquement de l’aorte dorsale droite distalement à la sixième artère intersegmentaire cervicale. Le diagnostic et la différentiation des anomalies de l’arc aortique est basée sur les trouvailles à la radiographie du thorax en association avec l’œsophagographie. Cette anomalie est habituellement asymptomatique. Lorsqu’elle devient symptomatique, elle produit une dysphagia lusoria ou une dyspnée et une toux chronique. Le traitement est indiqué pour améliorer la symptomatologie d’une dysphagia lusoria et pour la prévention de complications d’une dilatation anévrismale.

Introduction

The aberrant right subclavian artery is the most common anomaly of the aortic arch, occurring in 0.5 to 2.5% of individuals [1], [2], [3]. It is the first arch anomaly to have been described, in 1735 by Hunauld [4]. Inspired by Hommel's [5] description of a vascular ring formed by a double aortic arch as a lusus naturae (trick of nature), David Bayford was the first to describe dysphagia caused by an aberrant right subclavian artery [6], calling the clinical syndrome dysphagia lusoria and the aberrant artery causing it arteria lusoria.

With the advent and widespread use of precise non-invasive imaging techniques, such as computed tomography and magnetic resonance angiography, this arch anomaly is recognized more frequently. This review provides a concise overview of the epidemiology, development, anatomy, clinical presentation, imaging and management of arteria lusoria for the clinician confronted with a patient with this anomaly.

Section snippets

Developmental anatomy

The proximal subclavian artery derives from a complex succession of vascular segments resulting from the transformation of the six primordial paired branchial arches (Fig. 1a) [7], [8], [9]. It is surprising to note that the current understanding of the developmental anatomy of the aortic arch is based on the study of only 31 embryos. The following account is based mostly on the key work of E.D. Congdon of the Carnegie Institution in Washington. The segmental organization of the embryo

Anatomy

In the arteria lusoria configuration, four vessels arise sequentially from a left aortic arch: the right common carotid artery, the left common carotid artery, the left subclavian artery, and the aberrant right subclavian artery (Fig. 2). The latter arises then as the last branch of the aortic arch or from the proximal descending aorta, on the left side of the thorax and has to cross upwards and to the right either behind the esophagus (80–84%), between the esophagus and the trachea (12.7–15%),

Clinical picture of arteria lusoria

Arteria lusoria is usually asymptomatic, because the aberrant artery does not form a complete vascular ring around the esophagus and trachea, and is most often discovered during the course of evaluation of other mediastinal anomalies.

There are three settings in which an aberrant right subclavian artery becomes symptomatic:

•
when the esophagus and trachea are hemmed in between the lusorian artery dorsally and anteriorly by a truncus bicaroticus[21], [25];
•
from aberrant subclavian artery aneurysm;
•

Imaging

The diagnosis and differentiation of arch anomalies is based primarily on findings at chest radiography in association with those at esophagography. The lateral projection chest radiography can show the aberrant artery as a round, localized density continuous with the superior margin of the aortic arch. The anteroposterior projection can demonstrate a density in the mediastinum ascending obliquely from the superior margin of the aortic arch, although this may also be present in patients with

Treatment

Most patients with an aberrant right subclavian artery are asymptomatic and rarely warrant any treatment. Treatment is indicated for symptomatic relief of dysphagia lusoria, usually in children, although symptoms do not always disappear after correction. Treatment is also indicated for prevention of complications due to aneurysmal dilatation of the lusorian artery [35]. Conservative treatment of aneurysmal aberrant right subclavian artery is associated with high mortality and morbidity rates,

Conclusions

Arteria lusoria is the most common aortic arch anomaly. The diagnosis and differentiation of arch anomalies is based on findings at chest radiography in association with those at esophagography. The vascular anatomy and the relationship with the surrounding structures may be demonstrated with echocardiography, CT or MR angiography, although the performance of these different imaging techniques at reaching the diagnosis has not been evaluated. Occasionally, angiography may be required. A

Conflicts of interest

The authors report no conflict of interest or disclosures for this review.

Funding sources

No funding was necessary for this review.

References (60)

R.J. Moene et al.
Anatomic characteristics of ventricular septal defect associated with coarctation of the aorta
Am J Cardiol
(1987)
R. Krumlauf
Hox genes and pattern formation in the branchial region of the vertebrate head
Trends Genet
(1993)
P. Ramaswamy et al.
Frequency of aberrant subclavian artery, arch laterality, and associated intracardiac anomalies detected by echocardiography
Am J Cardiol
(2008)
P.D. Kiernan et al.
Aneurysm of an aberrant right subclavian artery: case report and review of the literature
Mayo Clin Proc
(1993)
R.J. Valentine et al.
Supraclavicular approach for the simultaneous treatment of dysphagia lusoria and thoracic outlet syndrome
Ann Vasc Surg
(1988)
D.L. Akers et al.
Complications of anomalous origin of the right subclavian artery: case report and review of the literature
Ann Vasc Surg
(1991)
R.G. Miller et al.
Survival after aberrant right subclavian artery-esophageal fistula: case report and literature review
J Vasc Surg
(1996)
R.J. Valentine et al.
A modified extrathoracic approach to the treatment of dysphagia lusoria
J Vasc Surg
(1987)
M.A. Gardner et al.
Aneurysms of an aberrant right subclavian artery: report of two cases
Heart Lung Circ
(2001)
H. Kamiya et al.
Surgical treatment of aberrant right subclavian artery (arteria lusoria) aneurysm using three different methods
Ann Thorac Surg
(2006)

J.N. Kokotsakis et al.

Surgical treatment for aneurysm at the origin of an aberrant right subclavian artery

Ann Vasc Surg

(2003)

R. Lee et al.

Elephant trunk reconstruction for aberrant right subclavian and aortic aneurysm

Ann Thorac Surg

(1997)

T. Ota et al.

Surgical treatment for Kommerell's diverticulum

J Thorac Cardiovasc Surg

(2006)

M. Davidian et al.

Aneurysm of an aberrant right subclavian artery: treatment with PTFE covered stent-graft

J Vasc Surg

(1998)

J.S. Corral et al.

Treatment of aberrant right subclavian artery aneurysm with endovascular exclusion and adjunctive surgical bypass

J Vasc Interv Radiol

(2003)

R.W. Simon et al.

Giant aneurysm of an aberrant right subclavian artery from the left aortic arch

J Thorac Cardiovasc Surg

(2006)

H. Hoppe et al.

Percutaneous treatment of aberrant right subclavian artery aneurysm with use of the Amplatzer septal occluder

J Vasc Interv Radiol

(2006)

J.E. Edwards

Anomalies of the aortic arch system

Birth Defects Orig Artic Ser

(1977)

G. Molz et al.

Aberrant subclavian artery (arteria lusoria): sex differences in the prevalence of various forms of the malformation. Evaluation of 1378 observations

Virchows Arch A Pathol Anat Histol

(1978)

H. Zapata et al.

Aberrant right subclavian artery with left aortic arch: associated cardiac anomalies

Pediatr Cardiol

(1993)

P.M. Hunauld

Examen de quelques parties d’un singe

Hist Acad Roy Sci

(1735)

Hommel D cited by Pointer CWM. Arterial anomalies pertaining to aortic arches and branches arising from them. Nebraska...

D. Bayford

An account on a singular case of obstructed deglutition

Memoirs Med Soc London

(1794)

E.D. Congdon

Transformation of the aortic arch system during the development of the human embryo

Contributions Embryol

(1922)

D.H. Padget

Development of cranial arteries in human embryo

Contributions Embryol

(1948)

D.H. Padget

Designation of the embryonic intersegmental arteries in reference to the vertebral artery and subclavian stem

Anat Rec

(1954)

A. Meeson et al.

A relationship between apoptosis and flow during programmed capillary regression is revealed by vital analysis

Development

(1996)

M.L. Kirby et al.

Neural crest and cardiovascular patterning

Circ Res

(1995)

H. Yanagisawa et al.

Role of Endothelin-1/Endothelin-A receptor-mediated signaling pathway in the aortic arch patterning in mice

J Clin Invest

(1998)

D.G. Molin et al.

Altered apoptosis pattern during pharyngeal arch artery remodelling is associated with aortic arch malformations in Tgfbeta2 knock-out mice

Cardiovasc Res

(2002)

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Aberrant right subclavian artery (ARSA) is a rare condition, but the most common anomaly of the aortic arch. Although neurointerventions via transradial access (TRA) are becoming increasingly popular worldwide, transradial carotid cannulation has been extremely challenging in patients with an ARSA. Herein, we present a case of ARSA-associated anterior communicating artery (ACoA) aneurysm that was successfully treated with a radial-specific 6F Simmons guiding sheath via left TRA. We also review the relevant literature.
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Transradial anterior circulation intervention has been rarely used for patients with an ARSA due to unfavorable catheter trajectory. Left TRA using the 6F Simmons guiding sheath is a useful treatment option to address anterior circulation interventions for patients with an ARSA. Preoperative diagnosis of ARSA is necessary for the application of our method.
A Simple Method to Diagnose Arteria Lusoria During Right Radial Access Coronary Intervention
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Arteria lusoria (aberrant right subclavian artery) occurs in approximately 0.1–2.4 % of all individuals. The resulting tortuosity can pose a challenge for coronary angiography using radial artery access, but also can aid in the diagnosis if not already established. This case series reports three patients diagnosed with arteria lusoria by a single low-volume catheterization operator over a 6-month period, noting that its prevalence may be higher than usually reported, can be suspected when a catheter from the right radial artery crosses the midline and forms a loop as it traverses to the ascending aorta, and that it does not preclude successful catheterization and coronary intervention.
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Esophageal dissection is generally safe and easy during transhiatal esophagectomy (THE). The right subclavian artery crosses between the esophagus and spine in about 1 % to 2 % of cases. This condition is called aberrant right subclavian artery and is the most common congenital aortic arch anomaly. Pre-operative recognition of this anomaly is important in esophageal surgeries. In unprepared situations injury to this vessel may result in life threatening bleeding.
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The presence of aberrant right subclavian artery causes difficulty in performing esophagectomy. If it is diagnosed preoperatively, cautious retroesophageal dissection prevents injury to this vessel and complications following it.
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Aberrant subclavian arteries (aSCAs), with or without aortic pathology, are uncommon. The purpose of the present study was to review our experience with the surgical management of aSCA.
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A total of 46 symptomatic and 3 asymptomatic patients with aSCA had undergone surgery (31 females [62%]; median age, 45 years). An aberrant right subclavian artery was present in 38 (78%) and an aberrant left subclavian artery in 11 patients (22%). Of the 49 patients, 41 (84%) had had a Kommerell diverticulum (KD) and 11 (22%) had had a concomitant distal arch or proximal descending thoracic aortic aneurysm. Symptoms included dysphagia (56%), dyspnea (27%), odynophagia (20%), and upper extremity exertional fatigue (16%). Five patients (10%) had required emergency surgery. The aSCA had been treated by transposition in 32, a carotid to subclavian bypass in 11, and an ascending aorta to subclavian bypass in 6. The KD was treated by resection and oversewing in 19 patients (39%). Fifteen patients (31%) had required distal arch or proximal descending thoracic aortic replacement for concomitant aortic disease and/or KD treatment. Thoracic endovascular aortic repair was used to exclude the KD in six patients (12%). Seven patients (14%) had undergone only bypass or transposition. The 30-day complications included one death from pulseless electrical activity arrest secondary to massive pulmonary embolism. The 30-day major complications (14%) included acute respiratory failure in three, early mortality in one, stroke in one, non–ST-elevation myocardial infarction in one, and temporary dialysis in one patient. The other complications included chylothorax/lymphocele (n = 5; 10%), acute kidney injury (n = 2; 4%), pneumonia (n = 2; 4%), wound infection (n = 2; 4%), atrial fibrillation (n = 2; 4%), Horner syndrome (n = 2; 4%), lower extremity acute limb ischemia (n = 1; 2%), and left recurrent laryngeal nerve injury (n = 1; 2%). At a median follow-up of 53 months (range, 1-230 months), 40 patients (82%) had had complete symptom relief and 9 (18%) had experienced improvement. Six patients had died at a median of 157 months; the deaths were not procedure or aortic related. The primary patency was 98%. Reintervention at ≤30 days had been required for two patients (4%) for ligation of lymphatic vessels and bilateral lower extremity fasciotomy after proximal descending thoracic aorta replacement. One patient had required late explantation of an infected and occluded carotid to subclavian bypass graft, which was treated by cryopreserved allograft replacement.
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Focus onArteria lusoria: Developmental anatomy, clinical, radiological and surgical aspectsArteria lusoria : embryologie, aspects cliniques, radiologiques et chirurgicaux

Abstract

Résumé

Introduction

Section snippets

Developmental anatomy

Anatomy

Clinical picture of arteria lusoria

Imaging

Treatment

Conclusions

Conflicts of interest

Funding sources

Am J Cardiol

Trends Genet

Am J Cardiol

Mayo Clin Proc

Ann Vasc Surg

Ann Vasc Surg

J Vasc Surg

J Vasc Surg

Heart Lung Circ

Ann Thorac Surg

Ann Vasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

J Vasc Surg

J Vasc Interv Radiol

J Thorac Cardiovasc Surg

J Vasc Interv Radiol

Anomalies of the aortic arch system

Birth Defects Orig Artic Ser

Aberrant subclavian artery (arteria lusoria): sex differences in the prevalence of various forms of the malformation. Evaluation of 1378 observations

Virchows Arch A Pathol Anat Histol

Aberrant right subclavian artery with left aortic arch: associated cardiac anomalies

Pediatr Cardiol

Examen de quelques parties d’un singe

Hist Acad Roy Sci

An account on a singular case of obstructed deglutition

Memoirs Med Soc London

Transformation of the aortic arch system during the development of the human embryo

Contributions Embryol

Development of cranial arteries in human embryo

Contributions Embryol

Designation of the embryonic intersegmental arteries in reference to the vertebral artery and subclavian stem

Anat Rec

A relationship between apoptosis and flow during programmed capillary regression is revealed by vital analysis

Development

Neural crest and cardiovascular patterning

Circ Res

Role of Endothelin-1/Endothelin-A receptor-mediated signaling pathway in the aortic arch patterning in mice

J Clin Invest

Altered apoptosis pattern during pharyngeal arch artery remodelling is associated with aortic arch malformations in Tgfbeta2 knock-out mice

Cardiovasc Res

Focus on
Arteria lusoria: Developmental anatomy, clinical, radiological and surgical aspectsArteria lusoria : embryologie, aspects cliniques, radiologiques et chirurgicaux