Vascular rings are uncommon congenital malformations that present with dysphagia or upper airway obstruction and are well described in the pediatric cardiology literature. Tracheal compression by the innominate artery is considered an “incomplete” vascular ring, and is a rare diagnosis of this category. The innominate artery crosses anterior to the trachea where it can cause compression and lead to stridor, respiratory distress, and recurrent lower respiratory tract infections. Typically symptoms are prominent in infancy but improve by the second year of life. To our knowledge, tracheal compression by the innominate artery has not been described presenting in adult population.
70-year-old West African woman presents to our hospital with dyspnea on exertion and left-sided chest pain. She is admitted for cardiac catheritization. Patient has been carrying a diagnosis of chronic obstructive pulmonary disease (COPD) for multiple years. She has had history of only second hand smoke exposure and has been treated with albuterol and ipratropium. Her other medical history included hypertension, hyperlipidemia, coronary arterial disease requiring coronary stent placement for acute myocardial infarction, kyphosis, and a ventral hernia. Her physical exam was notable for stridor, but polyphonic wheezing was not appreciated throughout. Furthermore, chest radiograph imaging was not consistent with the diagnosis of asthma or COPD. A high-resolution, multislice computed tomographic (CT) scan revealed a tortuous innominate artery indenting and significantly compressing the trachea. The patient had considerable kyphosis, which was thought to contribute to the tracheal compression presenting so late in life by causing changes in thoracic anatomy. The vascular surgery team was consulted. The patient was deemed a poor surgical candidate due to her advanced age and comorbidities, especially given the relatively stable nature of tracheal compression.
Tracheal compression from an innominate artery arising from a normal left-sided aortic arch is a rare phenomenon. It was first described by Gross and Neuhauser in 1948 in an infant presenting with cough, stridor and apnea. Numerous pediatric cases have since been reported, some with symptomatic relief after decompressive surgery. Strife et al found that up to 30% of children have some anterior indentation of the trachea without symptoms, and in one series only 39 of 285 symptomatic cases required surgery. Medical management includes humidified oxygen, steroids and antibiotics, while common surgical approaches include arteriopexy, with suspension of the innominate artery to the sternum, or reimplantation at a more proximal site of the ascending aorta. Typically symptoms of tracheal compression gradually improve in the first two years of life with the development of the trachea’s cartilaginous rings, and cephalic, rightward, anterior movement of the innominate artery away from the trachea. Myer et al reports of innominate artery compression of the trachea in adolescents, further suggesting that flow volume loops are an effective method for measuring compromise and that exercise intolerance and apnea spells are possible indications for surgical repair. Tracheal rings have rarely been newly diagnosed in adults. Several authors have reported them in adult patients presenting with a right aortic arch. However, we could not find any report of tracheal compression caused by the innominate artery presenting in adulthood.
This case of a 70-year-old woman with a rare tracheal compression demonstrates the importance to cast a wide differential when working up shortness of breath, especially when the history and physical do not match well with a common diagnosis. In this case an early correct diagnosis could have resulted in a possibility for a corrective decompressive vascular surgery with less morbidity.
Louis Gerolemou, No Financial Disclosure Information; No Product/Research Disclosure Information