{ "id": "epf9hefz9g", "slug": "right-aortic-arch", "title": "Right aortic arch", "updated": "2026-10-21", "topic_path": [ { "id": "life", "label": "Life and health", "api_url": "https://www.edgepedia.org/api/v1/topics/life" }, { "id": "life.health", "label": "Human health and medicine", "api_url": "https://www.edgepedia.org/api/v1/topics/life.health" }, { "id": "life.health.body", "label": "Human structure and function", "api_url": "https://www.edgepedia.org/api/v1/topics/life.health.body" }, { "id": "life.health.body.circulatory", "label": "Cardiovascular and lymphatic systems", "api_url": "https://www.edgepedia.org/api/v1/topics/life.health.body.circulatory" }, { "id": "life.health.body.circulatory.heart", "label": "Heart", "api_url": "https://www.edgepedia.org/api/v1/topics/life.health.body.circulatory.heart" }, { "id": "life.health.body.circulatory.congenital-structural-heart", "label": "Congenital and structural heart anomalies", "api_url": "https://www.edgepedia.org/api/v1/topics/life.health.body.circulatory.congenital-structural-heart" }, { "id": "life.health.body.circulatory.congenital-obstructive-anomalies", "label": "Congenital obstructive and connection anomalies", "api_url": "https://www.edgepedia.org/api/v1/topics/life.health.body.circulatory.congenital-obstructive-anomalies" }, { "id": "life.health.body.circulatory.congenital-obstructive-anomalies.vascular-rings-and-arch-anomalies", "label": "Vascular rings and aortic arch anomalies", "api_url": "https://www.edgepedia.org/api/v1/topics/life.health.body.circulatory.congenital-obstructive-anomalies.vascular-rings-and-arch-anomalies" } ], "geo": [ { "id": "geo.nongeo.nodate.life.health.body.circulatory.heart", "label": "Non-geographic · Not dated: Heart", "api_url": "https://www.edgepedia.org/api/v1/geo/geo.nongeo.nodate.life.health.body.circulatory.heart", "path": [ { "id": "geo.nongeo", "label": "Non-geographic", "api_url": "https://www.edgepedia.org/api/v1/geo/geo.nongeo" }, { "id": "geo.nongeo.nodate", "label": "Non-geographic · Not dated", "api_url": "https://www.edgepedia.org/api/v1/geo/geo.nongeo.nodate" }, { "id": "geo.nongeo.nodate.life", "label": "Life and health", "api_url": "https://www.edgepedia.org/api/v1/geo/geo.nongeo.nodate.life" }, { "id": "geo.nongeo.nodate.life.health", "label": "Human health and medicine", "api_url": "https://www.edgepedia.org/api/v1/geo/geo.nongeo.nodate.life.health" }, { "id": "geo.nongeo.nodate.life.health.body", "label": "Human structure and function", "api_url": "https://www.edgepedia.org/api/v1/geo/geo.nongeo.nodate.life.health.body" }, { "id": "geo.nongeo.nodate.life.health.body.circulatory", "label": "Cardiovascular and lymphatic systems", "api_url": "https://www.edgepedia.org/api/v1/geo/geo.nongeo.nodate.life.health.body.circulatory" }, { "id": "geo.nongeo.nodate.life.health.body.circulatory.heart", "label": "Heart", "api_url": "https://www.edgepedia.org/api/v1/geo/geo.nongeo.nodate.life.health.body.circulatory.heart" } ] } ], "excerpt": "Right-sided aortic arch (RAA), a congenital variant where the aortic arch courses right of the trachea, occurring in about 0.1% of people; main types are mirror-image branching and aberrant left subclavian artery, the latter forming a compressive vascular ring.", "snippet": "Right-sided aortic arch (RAA), a congenital variant where the aortic arch courses right of the trachea, occurring in about 0.1% of people; main types are mirror-image branching and aberrant left subclavian artery, the latter forming a compressive vascular ring.", "node": "life.health.body.circulatory.congenital-obstructive-anomalies.vascular-rings-and-arch-anomalies", "markdown": "# Right aortic arch\n\nA **right-sided aortic arch** (RAA) is a congenital variant of the aorta in which the aortic arch runs to the right and behind the trachea instead of its normal course to the left and in front. It occurs in about 0.1% of all human beings according to prenatal and adult population studies, and a meta-analysis estimated a pooled prevalence of 0.2%.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) • [2](https://pmc.ncbi.nlm.nih.gov/articles/PMC11172921/) Many people with the variant never have symptoms, but when the arch and its branch vessels together with a left-sided ligamentum arteriosum encircle the trachea and esophagus, the resulting [vascular ring](https://www.edgepedia.org/vascular-ring) can compress both structures and cause breathing and swallowing problems.[3](https://my.clevelandclinic.org/health/diseases/right-aortic-arch) • [1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n\n| Key fact | Detail |\n|---|---|\n| Prevalence | About 0.1% of humans in prenatal and adult studies; pooled prevalence 0.2% in a meta-analysis[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) • [2](https://pmc.ncbi.nlm.nih.gov/articles/PMC11172921/) |\n| Main types | RAA with aberrant left subclavian artery (~56% of patients) and RAA with mirror-image branching (~44%)[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) |\n| Vascular ring | The aberrant left subclavian type almost always forms a complete ring around the trachea and esophagus[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) |\n| Associated heart defects | Congenital heart disease in more than 75% of mirror-image cases, but fewer than 25% of the aberrant left subclavian type[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) |\n| Genetic association | 22q11 deletion syndrome in 5–19% of RAA cases, mostly in patients with intracardiac anomalies[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) |\n| Symptom onset | Respiratory symptoms usually begin between 6 and 12 months of age; digestive symptoms appear when solid foods start[3](https://my.clevelandclinic.org/health/diseases/right-aortic-arch) |\n| Surgical share | In a 515-patient surgical series, RAA with aberrant left subclavian artery and left ligamentum accounted for 62.7% of vascular ring repairs, double aortic arch for 33.8%[4](https://www.jacc.org/doi/10.1016/j.jacc.2024.05.078) |\n\n## Embryology and types\n\nThe aortic arch develops from six paired primitive arches connecting the ventral and dorsal aortas, which appear and regress in sequence during fetal development. Normally the right fourth arch involutes and the left fourth arch persists, producing a left-sided arch; a right-sided arch results from regression of the left fourth arch and persistence of the right.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n\nTwo major types are distinguished by where the left arch involuted relative to the head and neck branches.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n\n- **RAA with aberrant left subclavian artery.** The left subclavian artery arises last from the arch, usually from an outpouching called a Kommerell's diverticulum, and courses behind the esophagus. This arrangement occurs in about 56% of patients and almost inevitably forms a complete vascular ring.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n- **RAA with mirror-image branching.** The branches leave the arch in the mirror image of normal anatomy, with a left innominate artery giving rise to the left common carotid and left subclavian arteries, in roughly 44% of patients. A ring forms only when the ligamentum arteriosum stretches from the left descending aorta to the pulmonary artery; more often it originates from the innominate artery, producing only a partial ring.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n\nA **Kommerell's diverticulum**, first described by Burckhard Kommerell in 1936, is an aneurysmal dilation at the base of the aberrant subclavian artery and an independent source of tracheoesophageal compression.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) • [5](https://brieflands.com/articles/jcp-156671.pdf)\n\n## Symptoms\n\nSymptoms depend on whether the arch forms a vascular ring. Without a ring, or with a loose partial ring, a child may have no symptoms at all. When a complete ring squeezes the trachea and esophagus, typical features include cough, noisy breathing (stridor), wheezing, shortness of breath with activity, recurrent respiratory tract infections, nausea and vomiting, reflux, and trouble swallowing solid foods.[3](https://my.clevelandclinic.org/health/diseases/right-aortic-arch)\n\nRespiratory symptoms usually start between 6 and 12 months of age and often worsen when the baby flexes the neck and improve with neck extension, because flexion narrows the already compressed airway. Digestive problems rarely appear until the infant transitions from milk to solid food; in older children, an unusually slow pace of eating may be the only sign.[3](https://my.clevelandclinic.org/health/diseases/right-aortic-arch) • [1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) Symptoms are often subtle and mimic asthma, bronchiolitis, [laryngomalacia](https://www.edgepedia.org/laryngomalacia), or reflux, which delays diagnosis.[6](https://www.cambridge.org/core/services/aop-cambridge-core/content/view/101C8D89EFAD9C234DD6B16851D497C1/S1047951125001544a.pdf/vascular_rings_what_has_changed_and_what_do_i_need_to_know_as_a_practitioner.pdf)\n\nAdults diagnosed later may present with chest, back, or shoulder discomfort related to atherosclerotic change in the aberrant vessels or to enlargement, dissection, or rupture of a Kommerell's diverticulum.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n\n## Associated conditions\n\n**Congenital heart disease** is common in the mirror-image type, occurring in more than 75% of cases, typically conotruncal (heart defects of the outflow tract region) and complex cyanotic anomalies such as tetralogy of Fallot, common arterial trunk, double-outlet right ventricle, [pulmonary atresia with ventricular septal defect](https://www.edgepedia.org/pulmonary-atresia-with-ventricular-septal-defect), and atrioventricular septal defect. In the aberrant left subclavian type, intracardiac malformations occur in fewer than 25% of cases, most often isolated septal defects or transposition of the great arteries.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n\n**Genetic abnormalities** have been reported in 9% to 34% of prenatal and postnatal RAA cohorts. The 22q11 deletion syndrome ([DiGeorge syndrome](https://www.edgepedia.org/digeorge-syndrome)) occurs in 5–19% of RAA cases, mostly in patients with intracardiac anomalies; when the two subtypes are compared, 22q11 deletions are found in 0–12% of patients with an aberrant left subclavian artery and in 13–17% of those with mirror-image branching.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) A right-sided aortic arch demonstrated by any modality should prompt consideration of evaluation for DiGeorge syndrome.[6](https://www.cambridge.org/core/services/aop-cambridge-core/content/view/101C8D89EFAD9C234DD6B16851D497C1/S1047951125001544a.pdf/vascular_rings_what_has_changed_and_what_do_i_need_to_know_as_a_practitioner.pdf)\n\n## Diagnosis\n\nA chest X-ray can show which side the aortic arch is on and may reveal tracheal changes suggesting a ring.[7](https://www.mayoclinic.org/diseases-conditions/vascular-rings/cdc-20389579) Barium swallow studies, once the primary imaging tool, show indentation of the esophagus but have increasingly been replaced by computed tomography angiography and magnetic resonance imaging, which delineate the full ring configuration; CT requires intravenous contrast and radiation, so some centers prefer MRI.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) Echocardiography is of limited value for confirming the ring itself because of poor acoustic windows and inability to visualize atretic segments, but it is valuable for detecting associated intracardiac anomalies.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) [Bronchoscopy](https://www.edgepedia.org/bronchoscopy) assesses the degree of airway obstruction and rules out other causes such as tracheomalacia.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n\nMany cases are now found before birth. In one fetal series of 71 right [aortic arches](https://www.edgepedia.org/aortic-arches), 37% had an aberrant left subclavian artery, 32% mirror-image branching, 28% an unknown type, and 3% a double aortic arch.[8](https://onlinelibrary.wiley.com/doi/abs/10.1002/uog.3883) A right arch with recurrent upper respiratory infections or any swallowing symptoms should prompt CT scanning, but in an asymptomatic patient further workup may be deferred.[6](https://www.cambridge.org/core/services/aop-cambridge-core/content/view/101C8D89EFAD9C234DD6B16851D497C1/S1047951125001544a.pdf/vascular_rings_what_has_changed_and_what_do_i_need_to_know_as_a_practitioner.pdf)\n\n## Treatment and outcomes\n\n**Conservative management** is reasonable for asymptomatic or mildly symptomatic patients. A review of the natural history concluded that watchful waiting is safe regardless of arch configuration, and mild signs of tracheoesophageal compression often improve and may disappear by age 4.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) In a cohort of 90 prenatally diagnosed aortic arch anomalies, 41 of 57 survivors (77.4%) had a vascular ring or sling, but only two developed symptoms and one needed an operation, leading the authors to conclude that intervention for vascular ring is rarely necessary.[9](https://pubmed.ncbi.nlm.nih.gov/38337754/) A meta-analysis cited in the surgical literature found that 83% of fetuses with prenatally detected RAA with aberrant left subclavian artery and left ligamentum remained free from ring-related surgery in the first 2 years of life.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n\n**Surgery** is indicated once moderate-to-severe symptoms develop. The ring is released by dividing the ductus or ligamentum arteriosum, usually through a left thoracotomy without cardiopulmonary bypass in children, or through median sternotomy when intracardiac repair is also needed. When a Kommerell's diverticulum exceeds 1.5 times the diameter of the subclavian artery, many surgeons resect it at the initial operation and reimplant the aberrant left subclavian artery onto the carotid artery, because a residual diverticulum can cause recurrent symptoms years later.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) In adults, surgery is usually driven by progressive enlargement, dissection, or rupture of the diverticulum rather than by the ring itself, since adult tracheal narrowing may have become fixed.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n\n**Outcomes** are generally good. Postoperative morbidity and mortality are low and relate more to concomitant cardiac and extracardiac anomalies than to the procedure itself; most patients have full resolution of symptoms within months to years, although a subset retains tracheobronchial narrowing and occasionally needs reintervention.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044) In a 515-patient single-institution series of complete vascular ring repairs spanning 1990 to 2023, RAA with aberrant left subclavian artery and left ligamentum was the most common diagnosis (62.7%), and 19.6% of patients had a genetic syndrome.[4](https://www.jacc.org/doi/10.1016/j.jacc.2024.05.078)\n\n## History\n\nThe first surgical division of a vascular ring was performed by Robert E. Gross at [Boston Children's Hospital](https://www.edgepedia.org/boston-childrens-hospital) in 1945, on an infant with a double aortic arch; Gross's report also described the entity now known as right aortic arch with left ductus, and he began operating on these arches in 1955. Willis J. Potts at Children's Memorial Hospital in Chicago started one of the largest early series of vascular ring surgery at the same time.[1](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n\n## References\n\n1. [Biermann D, Holst T, Hüners I, et al. Right aortic arch forming a true vascular ring: a clinical review. European Journal of Cardio-Thoracic Surgery 2021;60(5):1014–1021.](https://academic.oup.com/ejcts/article/60/5/1014/6273044)\n2. [Right-Sided Aortic Arch: A Computed Tomography Angiography Investigation, A Systematic Review with Meta-Analysis. PMC.](https://pmc.ncbi.nlm.nih.gov/articles/PMC11172921/)\n3. [Right Aortic Arch: Types, Symptoms & Treatment. Cleveland Clinic.](https://my.clevelandclinic.org/health/diseases/right-aortic-arch)\n4. [Outcomes of Patients Undergoing Surgery for Complete Vascular Rings. Journal of the American College of Cardiology.](https://www.jacc.org/doi/10.1016/j.jacc.2024.05.078)\n5. [Right Aortic Arch in Pediatric: Experience of a Center. Journal of Clinical and Basic Cardiology.](https://brieflands.com/articles/jcp-156671.pdf)\n6. [Madira S, et al. Vascular rings – what has changed, and what do I need to know as a practitioner? Cardiology in the Young 2025;35:881–887.](https://www.cambridge.org/core/services/aop-cambridge-core/content/view/101C8D89EFAD9C234DD6B16851D497C1/S1047951125001544a.pdf/vascular_rings_what_has_changed_and_what_do_i_need_to_know_as_a_practitioner.pdf)\n7. [Vascular rings – Overview. Mayo Clinic.](https://www.mayoclinic.org/diseases-conditions/vascular-rings/cdc-20389579)\n8. [Berg C, et al. Right aortic arch detected in fetal life. Ultrasound in Obstetrics & Gynecology 2006.](https://onlinelibrary.wiley.com/doi/abs/10.1002/uog.3883)\n9. [Associated Anomalies and Outcome in Patients with Prenatal Diagnosis of Aortic Arch Anomalies. PubMed.](https://pubmed.ncbi.nlm.nih.gov/38337754/)\n\n---\n*Topic: Encyclopedia › Life and health › Human health and medicine › Human structure and function › Cardiovascular and lymphatic systems › Heart › Congenital and structural heart anomalies › Congenital obstructive and connection anomalies › Vascular rings and aortic arch anomalies*\n\n*Initially written Sep 17, 2026 · Reviewed: Oct 21, 2026 · Edited: Oct 15, 2026; Oct 21, 2026 · Last review: Oct 21, 2026*\n\n*Copyright 2026 EdgeChat AI, a subsidiary of Biostate AI.*\n\nLicense: Edgepedia Community License 1.0, https://www.edgepedia.org/edgepedia/license\n", "same_as": [], "url": "https://www.edgepedia.org/right-aortic-arch", "markdown_url": "https://www.edgepedia.org/right-aortic-arch.md", "license": { "name": "Edgepedia Community License 1.0", "url": "https://www.edgepedia.org/edgepedia/license", "summary": "Free with credit, commercial use included. AI training is open to everyone. For other uses, organizations over USD 100M in revenue or 100M monthly users license separately.", "spdx": "LicenseRef-Edgepedia-Community-1.0" }, "credit": "\"Right aortic arch\", Edgepedia (EdgeChat), https://www.edgepedia.org/right-aortic-arch. Edgepedia Community License 1.0.", "credit_md": "\"[Right aortic arch](https://www.edgepedia.org/right-aortic-arch)\", Edgepedia (EdgeChat), [https://www.edgepedia.org/right-aortic-arch](https://www.edgepedia.org/right-aortic-arch). [Edgepedia Community License 1.0](https://www.edgepedia.org/edgepedia/license).", "credit_html": "\"Right aortic arch\", Edgepedia (EdgeChat), https://www.edgepedia.org/right-aortic-arch. Edgepedia Community License 1.0.", "speakable": "Right-sided aortic arch, a congenital variant where the aortic arch courses right of the trachea, occurring in about 0.1% of people; main types are mirror-image branching and aberrant left subclavian artery, the latter forming a compressive vascular ring." }