Working Diagnosis:
Right-sided aortic arch with aberrant left subclavian artery resulting in tracheomalacia secondary to vascular ring.

Treatment:
The patient was referred to cardiothoracic surgery and underwent division of the vascular ring with translocation of the aberrant left subclavian artery.
The Kommerell diverticulum was oversewn at the base.

Outcome:
At her two-week follow-up, she was noted to be recovering well and walking without shortness of breath.

Approximately six weeks after surgery she began cardiac rehabilitation.

Three months after surgery, she graduated from her academic program and was subsequently lost to follow up.

Author's Comments:
Right-sided aortic arch is a rare congenital anomaly, affecting 0.01-0.1% of the population1,2. It is classified into three types, first described by Dr. Jesse Edwards, based on the developmental pattern of the major head and neck vessels Case Photo #6 3. The most common type (50%) is mirror-image branching of the major vessels, followed by an aberrant left subclavian artery creating a fourth vascular branch from the aorta (40%), and finally obliteration of the left subclavian artery with collateralization (1.5%).

Mirror image anomalies are often associated with other congenital conotruncal malformations, such as Tetralogy of Fallot, truncus arteriosus, and transposition of the great arteries. Right-sided aortic arch is also associated with DiGeorge syndrome (22q11 deletion)2.In the absence of additional cardiac anomalies, isolated mirror-image right aortic arch is generally benign. In contrast, an aberrant left subclavian artery may contribute to the formation of a vascular ring, particularly due to the course of the ligamentum arteriosum, potentially causing tracheal or esophageal stricture. CT angiography is the preferred imaging modality to delineate vascular anatomy, guide risk assessment, and assist in surgical planning 4. Surgical correction of vascular rings may be necessary in symptomatic cases presenting with dyspnea or dysphagia.

Kommerell diverticulum is an anatomical anomaly often associated with an aberrant take-off of the left subclavian artery, that results in formation of a bulbous aneurysmal diverticulum at the arterial origin. It is present in 20-60% of people with aberrant subclavian arteries 5. These can cause dyspnea, dysphagia, or chest discomfort if the bulb is large enough to compress nearby structures. Major complications include rupture or dissection of the diverticulum. Surgical intervention is considered if the diameter of the orifice exceeds 30 mm4. There is no singularly agreed-upon treatment option, but these may include thoracic endovascular stent-graft replacement or bypass of the aberrant artery 6-8.

Editor's Comments:
Exertional respiratory symptoms are common in athletes, but this case highlights an uncommon vascular cause. Right-sided aortic arch and Kommerell diverticula are rare congenital abnormalities, occurring twice as often in males. Kommerell diverticulum (KD) can form a vascular ring, leading to symptoms such as dysphagia, chest pain, shortness of breath, hoarseness, and wheezing, due to tracheal compression. KD may also present with blood pressure discrepancies or asymmetric upper extremity pulses. Surgery is recommended for symptomatic patients and generally has high success rates, though some may continue to experience symptoms postoperatively. A multidisciplinary approach is essential, especially when considering return to sport, as there are no clear guidelines for post-surgical clearance.Vascular causes should be considered when respiratory symptoms persist despite treatment.

References:
1. Law MA, Mohan J. Right Aortic Arches. In: StatPearls. Treasure Island (FL) companies. Disclosure: Jay Mohan declares no relevant financial relationships with ineligible companies.2025.
2. McElhinney DB, Tworetzky W, Hanley FL, Rudolph AM. Congenital obstructive lesions of the right aortic arch. The Annals of Thoracic Surgery. 1999;67(4):1194-1202.
3. Knight L, Edwards JE. Right Aortic Arch. Circulation. 1974;50(5):1047-1051.
4. Tanaka A, Milner R, Ota T. Kommerells diverticulum in the current era: a comprehensive review. General Thoracic and Cardiovascular Surgery. 2015;63(5):245-259.
5. Niknejad MT. Right-sided aortic arch with aberrant left subclavian artery and Kommerell diverticulum. Radiopaedia. 2022.
6. An K, Li S, Yan J, Wang X, Hua Z. Translocation of aberrant left subclavian artery and resection of Kommerell diverticulum during the concomitant repair of intracardiac anomalies. Interactive CardioVascular and Thoracic Surgery. 2020;32(1):118-121.
7. Solano A, Pizano A, Azam J, et al. Kommerell's Diverticulum in a Right-Sided Aortic Arch With an Aberrant Left Subclavian Artery Hybrid Repair. Vasc Endovascular Surg. 2023;57(8):954-959.
8. Isselbacher EM, Bloom JP. Aberrant Subclavian Artery in Heritable Aortopathies. JACC. 2023;81(10):992-993.

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