P3C065: The Patient's All Right: An Adult Presentation of a Congenital Aortic Anomaly

Introduction: The differential diagnosis for chronic dyspnea in adult patients is likely to include common or frequently taught ailments such as anemia, asthma, or cardiac dysfunction. Congenital anomalies of the aorta are less likely to be considered, likely due to the rarity of these conditions as well as pediatric onset of symptoms. This case demonstrates an adult presentation of a vascular ring created by a right sided aortic arch with aberrant left subclavian artery.

Case Description: A 27 year old previously healthy female presented to establish care with a new primary care physician with the chief complaint of shortness of breath. In the last 6 months, she started a new position which requires long periods of presenting and she notes that at the end of presentations she feels like she is gasping for air. Prior to onset of subjective sensation of shortness of breath, she had been told that she appears to “gasp for air” at the end of long sentences or when speaking quickly. This had been pointed out to her intermittently over the last 3 years, however 2 weeks prior to presentation she heard a video of herself speaking and noted the gasping. There is no associated chest pain, palpitations, cough, no leg swelling, no orthopnea or paroxysmal nocturnal dyspnea. Chest radiograph is obtained which shows clear lung fields, normal cardiac silhouette, and incidental right sided aortic arch. Pulmonary function tests showed flattening of both inspiratory and expiratory curves, consistent with fixed airway obstruction. Follow up chest computed tomography demonstrated a right-sided aortic arch with aberrant left subclavian artery creating a vascular ring with slight indentation of trachea. The patient was referred to congenital cardiologist as well as pediatric cardiothoracic surgeon for consideration of repair.

Discussion: This case demonstrates adult presentation of a congenital aortic arch anomaly. Right-sided aortic arch with aberrant left subclavian artery is the most common form of right aortic arch and is typically asymptomatic in adults. The vascular ring is created by the arch crossing over the trachea and is completed by the left ligamentum arteriosum arising from the remnant of the left 4th aortic arch. Vascular rings and slings, while frequently on the differential for pediatric patients, are less likely to be considered in an adult patient in lieu of more common or well-described processes such as asthma, heart failure, anemia, or deconditioning. Initial workup for chronic dyspnea including chest radiographs, pulmonary function testing, and computed tomography will likely detect congenital aortic arch anomalies.

Conclusion: Congenital aortic arch anomalies can be considered as part of the differential diagnosis for adult patients presenting with chronic dyspnea.