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Cardiovascular Disease |

Case of a Septuagenarian With an Asymptomatic Double Aortic Arch

Aarti Mittal, DO; Ching-Fei Chang, MD
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University of Southern California, Division of Pulmonary, Critical Care, and Sleep Medicine, Los Angeles, CA


Chest. 2013;144(4_MeetingAbstracts):125A. doi:10.1378/chest.1702841
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Abstract

SESSION TITLE: Cardiovascular Case Report Posters II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: A double aortic arch (DAA) is a rare congenital anomaly where the ascending aorta divides into two branches, encircling the trachea and esophagus then conjoining again to form a single descending aorta. We report a case of an elderly patient with balanced-type DAA, found incidentally during the evaluation for pneumonia.

CASE PRESENTATION: A 71-year-old female with rheumatoid arthritis presented to the emergency room with a one week history of shortness of breath and productive cough. On chest x-ray, she was found to have pulmonary nodules and an extra mogul in her superior mediastinum. Chest CT scan revealed an ascending aorta which divided into twin arches forming a complete vascular ring around the trachea and esophagus, but without radiographic evidence of compression. Bronchoscopy determined that the nodules were from rheumatoid lung disease. Due to her otherwise asymptomatic status, the patient was sent home on empiric antibiotics and recovered.

DISCUSSION: DAA occurs in less than 1% of all cases of congenital heart disease. Approximately 200 cases exist in the literature thus far, but the average age at diagnosis is between 6 months to 2.5 years old. Most cases are discovered during the workup of symptoms related to tracheal or esophageal compression. Diagnosis is confirmed with CT or MRI scanning which demonstrates a classic 4-vessel sign in the superior mediastinum due to separate subclavian and common carotid arteries on both sides. There are three subtypes of DAA, depending on the relative sizes of the two arches: right-dominant (75%), left-dominant (20%), and balanced-type (5%). Interestingly, the dual-sidedness of balanced-type DAA causes the descending aorta to run more midline, resulting in abnormal stacking of structures anterior to the spine and compression of the left mainstem bronchus. Surgical correction is reserved for symptomatic patients only, but may still leave residual respiratory deficits in up to 54% of patients, mostly due to tracheobronchomalacia. No treatment is necessary for asymptomatic cases.

CONCLUSIONS: We report a very rare case of a septuagenarian who has lived her entire life with an undiagnosed balanced-type double aortic arch that remains relatively asymptomatic.

Reference #1: http://www.ctsnet.org/sections/clinicalresources/clinicalcases/article-23.html

DISCLOSURE: The following authors have nothing to disclose: Aarti Mittal, Ching-Fei Chang

No Product/Research Disclosure Information


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