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Rare Congenital Abnormality as a Cause of Dyspnea in an Adult FREE TO VIEW

Shenil Shah, MD; Carlos A. Jamis-Dow, MD; Margaret Wojnar, MD
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Pulmonary and Critical Care, Penn State Milton S. Hershey Medical Center, Hummelstown, PA

Chest. 2014;146(4_MeetingAbstracts):929A. doi:10.1378/chest.1992927
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SESSION TITLE: Miscellaneous Cases II

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Monday, October 27, 2014 at 03:15 PM - 04:15 PM

INTRODUCTION: Incomplete vascular rings are rare congenital vascular anomalies that may cause tracheal compression. It is even more unusual for these anomalies to be found during adulthood. We present an interesting case of a 20-year-old female presenting with dyspnea initially diagnosed and treated for asthma, but ultimately found to have a right sided aortic arch, vascular ring, and tracheal compression as the cause of her symptoms.

CASE PRESENTATION: KD presented to our pulmonary clinic with a 5 month history of a non-productive cough, dyspnea and wheezing requiring frequent use of her rescue inhaler. She had a past history of childhood asthma, but was asymptomatic over the previous 8 years. Review of systems was positive for lifelong exertional limitations.

DISCUSSION: Pulmonary Function Testing was performed (% predicted): FVC-2.41 (58%), FEV1-1.87 (52%), FEV1/FVC-77%. A significant response to bronchodilator was observed. TLC-3.42 (62%), RV-0.87 (66%), RV/TLC-26%, and DLCO-18.0 (56%). A chest XRay prior to her clinic visit originally showed no abnormalities, but was later finalized as showing a right aortic arch indenting the trachea. Her low TLC and DLCO were inconsistent findings with her diagnosis of asthma, prompting a high resolution chest CT that demonstrated a right aortic arch surrounding approximately 270 degrees of the trachea and causing 55% focal narrowing of the trachea. The left carotid, right carotid, right subclavian, and left subclavian arteries arose in that order from the right aortic arch. The left carotid artery coursed anterior to the trachea. She was referred to the Adult Congenital Heart Disease clinic, who ordered a Cardiac MRI that showed a right aortic arch with diverticulum of Kommerell, retroesophageal left subclavian artery and the aforementioned tracheal narrowing. A Transthoracic Echocardiogram showed normal LV size, EF 60%, no LVH, valvular abnormalities, or wall motion abnormalities. Given these findings, she underwent surgical correction with complete resolution of her dyspnea.

CONCLUSIONS: Dyspnea and exercise limitations are common presentations of pulmonary disorders. However, this case demonstrated a rare congenital cause of her symptoms. This serves to remind us that when symptoms and objective evidence contradict, further investigation is warranted.

Reference #1: Bakker DA, Berger RM, Witsenburg M, Bogers AJ. Vascular rings: a rare cause of common respiratory symptoms. Acta Paediatr 1999;88:947.

DISCLOSURE: The following authors have nothing to disclose: Shenil Shah, Carlos A. Jamis-Dow, Margaret Wojnar

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