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(Chest. 2004;126:1694-1697.)
© 2004 American College of Chest Physicians

Dysphagia, Chest Pain, and Refractory Asthma in a 42-Year-Old Woman*

Robert A. Winn, MD; Edward D. Chan, MD; Esther L. Langmack, MD, FCCP; Chakradhar Kotaru, MD and Elizabeth Aronsen, MD

* From the Division of Pulmonary Sciences and Critical Care Medicine (Dr. Aronsen), University of Colorado Health Sciences Center, Denver; Denver Veterans Affairs Medical Center (Dr. Winn), Denver; and National Jewish Medical and Research Center (Drs. Chan, Langmack, and Kotaru), Denver, CO.

Correspondence to: Robert A. Winn, MD, University of Colorado Health Sciences Center, Division of Pulmonary Sciences and Critical Care Medicine, Campus Box C272, 4200 E Ninth Ave, Denver, CO 80262; e-mail: robert.winn{at}uchsc.edu


   Introduction
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 Introduction
What is the cause...
 References
 
A 42-year-old woman who had a diagnosis of asthma since childhood that was refractory to corticosteroid therapy complained of chest discomfort, progressive dyspnea on exertion, and chronic cough, all gradually worsening in severity over the past several years. Her chest pain was described as a constant band-like pressure encircling the chest wall, and was exacerbated by cold weather, exertion, supine position, and forced expiration. The chest pain occasionally radiated to the throat and left shoulder. Both the chest pain and dyspnea were partially relieved by inhaled ß-agonist, nitroglycerin, and/or rest. In addition, she complained of intermittent dysphagia for solids and liquids. Her medications included a short-acting ß2-agonist, inhaled corticosteroids, and intermittent oral corticosteroids.

Results of her physical examination were within normal limits, except for mild Cushingnoid features. Routine laboratory blood studies were unremarkable. A chest radiograph is shown in Figure 1 . Findings on ECG, cardiac enzymes during a severe episode of chest pain, two-dimensional echocardiogram, cardiac catheterization, and a 24-h ambulatory pH probe were all within normal limits. Spirometry showed a FVC of 3.67 L (102% of predicted), FEV1 of 2.96 L (99% of predicted), and FEV1/FVC ratio of 81%. The flow-volume loop showed flattening of the expiratory segment (Fig 2 ).



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  		Figure 1.. The chest radiographs of a 42-year-old women with asthma-like symptoms revealing a right-sided aortic arch and distal trachea narrowing.

 


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  		Figure 2.. Flow-volume loop demonstrating flattening of the expiratory portion, suggesting variable intrathoracic obstruction. Pred = predicted.

 

   What is the cause of this patient’s chest pain, dyspnea, and dysphagia?
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 What is the cause...
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Diagnosis: Vascular ring leading to symptomatic tracheoesophageal compression
The chest radiograph showed a right-sided aortic arch and distal tracheal indentation (Fig 1). A thoracic CT scan also revealed a right-sided aortic arch with a right descending thoracic aorta, a fibrous band, and an aberrant left subclavian artery (Fig 3 ). In addition, a retroesophageal diverticulum produced anterior displacement and compression of the trachea and esophagus. Bronchoscopy revealed tracheal compression 3 cm above the carina and extending proximally 3 cm in length. Because of persistent symptoms from the vascular ring, a left posterolateral thoracotomy through the fourth intercostal space was performed. At surgery, the ligamentum arteriosum was identified as a thick, fibrous band compressing both the esophagus and trachea, and an aberrant left subclavian artery was also seen traveling behind the esophagus. The band was divided. Postoperatively, the patient had immediate resolution of her chest pain and dyspnea. Her dysphagia also improved. She has remained asymptomatic since the operation, and has not required corticosteroids or any other asthma medications.



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  		Figure 3.. Chest CT scan with IV contrast showing a vascular ring. The right-sided ascending (A) and descending (D) thoracic aorta, and an aberrant left subclavian artery (arrow) are demonstrated.

 
A vascular ring is a rare congenital abnormality that can cause complete or partial obstruction of the esophagus and/or trachea. A complete vascular ring is formed by a right aortic arch, left ligamentum arteriosum that fails to regress, and aberrant left subclavian artery. Vascular rings occur with an incidence of < 0.2%, and usually presents in infancy or childhood with respiratory symptoms consisting of wheeze, recurrent respiratory tract infection, or stridor.12 The mean age at onset of symptoms ranges from 7 months to 4.5 years.3 GI symptoms such as emesis and/or dysphagia occurred in approximately 40% of cases.3 Most patients with vascular rings will require surgical repair.4 Tracheoesophageal compression has been described in a large series of infants and children,56 but documented cases in adults are rare.2 While the incidence of vascular rings in adults is not known, the occurrence of right-sided aortic arch, an anomaly associated with a higher likelihood of a complete vascular ring, is approximately 0.1%.267 In adults, either swallowing or respiratory symptoms may predominate with the presence of a vascular ring.2

The right aortic arch with ligamentum arteriosum form of vascular ring causes between 25% and 30% of all cases of vascular rings.1 In fact, it is the second most common type of vascular ring requiring surgery, occurring in an estimated 1 in 1,000 individuals in the general population.8 In this type of abnormality, as seen in our patient, the aortic arch is to the right of the trachea. The ligamentum arteriosum extends from the main pulmonary artery to the descending thoracic aorta, completing the ring.9 The right aortic arch with ligamentum arteriosum form of vascular ring results from a persistent right fourth brachial arch and an absent left arch, which is interrupted between the left common carotid and left subclavian artery.510 Isolated aortic arch abnormalities have been associated with chromosome 22q11 deletion,111213 and > 75% of patients with this chromosomal abnormality have congenital cardiovascular defects.

The respiratory symptoms associated with this anomaly may result from either static or dynamic changes in the trachea, bronchi, lungs, and thoracic cage.2 Vascular rings tend to become more constrictive as the trachea grows.14 The tracheomalacia may diminish once the compression is relieved.1 Symptoms may occur transiently with exertion, supine positioning, and fluid administration that dilates the aorta.1516 Superimposed illnesses, such as asthma or respiratory tract infections, may cause further increases in airway resistance exacerbating airflow obstruction.2 In addition, gastroesophageal reflux due to the esophageal compression from the vascular ring may also contribute to the respiratory symptoms.17

The diagnostic workup for this anomaly should include a chest radiograph, which will often show a right-sided aortic arch, a distal tracheal indentation on the posterolateral view, and a retrotracheal opacity or anterior tracheal bowing on lateral view.18 A barium swallow may also show compression of the esophagus.1920 Additional studies that might be helpful include a thoracic CT scan or MRI,21 and an aortogram. Two-dimensional echocardiography has been reported to be insensitive in the diagnosis of vascular rings.22 The value of bronchoscopy is primarily to rule out other anatomic causes of airway obstruction, including endobronchial neoplasm or airway stenosis.2 Spirometry findings may be normal or may show flattening of the expiratory portion of the flow-volume loop, suggesting a variable intrathoracic obstruction.152324 The diffusion capacity is typically normal.1524

Surgical division of the ligamentum arteriosum is the treatment of choice in symptomatic patients. In those who continue to have persistent symptoms despite surgery, tracheal resection or intraluminal stenting may be necessary.1

Vascular rings should be included in the differential diagnosis of all adult patients with dyspnea and/or dysphagia accompanied by an aortic arch anomaly. Diagnosis of this condition in patients with chronic, nonspecific respiratory complaints may eliminate the need for corticosteroids and other respiratory medications. A high degree of clinical suspicion is required for the recognition of this rare disease entity.

Received for publication January 9, 2004. Accepted for publication January 22, 2004.


   References
TOP
 Introduction
 What is the cause...
 References
 

  1. van Son, JA, Julsrud, PR, Hagler, DJ, et al (1993) Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clin Proc 68,1056-1063[ISI][Medline]
  2. Grathwohl, KW, Afifi, AY, Dillard, TA, et al Vascular rings of the thoracic aorta in adults. Am Surg 1999;65,1077-1083[ISI][Medline]
  3. Kocis, KC, Midgley, FM, Ruckman, RN Aortic arch complex anomalies: 20-year experience with symptoms, diagnosis, associated cardiac defects, and surgical repair. Pediatr Cardiol 1997;18,127-132[CrossRef][ISI][Medline]
  4. Backer, CL, Ilbawi, MN, Idriss, FS, et al Vascular anomalies causing tracheoesophageal compression: review of experience in children. J Thorac Cardiovasc Surg 1989;97,725-731[Abstract]
  5. Adkins, RB, Jr, Maples, MD, Graham, BS, et al Dysphagia associated with an aortic arch anomaly in adults. Am Surg 1986;52,238-245[ISI][Medline]
  6. Stewart, JR, Kincaid, OW, Titus, JL Right aortic arch: plain film diagnosis and significance. AJR Am J Roentgenol Radium Ther Nucl Med 1966;97,377-389[ISI][Medline]
  7. Hastreiter, AR, D’Cruz, IA, Cantez, T, et al Right-sided aorta. I. Occurrence of right aortic arch in various types of congenital heart disease. II. Right aortic arch, right descending aorta, and associated anomalies. Br Heart J 1966;28,722-739[Free Full Text]
  8. McNally, PR, Rak, KM Dysphagia lusoria caused by persistent right aortic arch with aberrant left subclavian artery and diverticulum of Kommerell. Dig Dis Sci 1992;37,144-149[CrossRef][ISI][Medline]
  9. Backer, CL, Mavroudis, C Heart Surgery Nomenclature and Database Project: patent ductus arteriosus, coarctation of the aorta, interrupted aortic arch. Ann Thorac Surg 2000;69,S298-S307[Abstract/Free Full Text]
  10. Morris, CD, Kanter, KR, Miller, JI, Jr Late-onset dysphagia lusoria. Ann Thorac Surg 2001;71,710-712[Abstract/Free Full Text]
  11. Goldmuntz, E, Clark, BJ, Mitchell, LE, et al Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol 1998;32,492-498[Abstract/Free Full Text]
  12. McElhinney, DB, McDonald-McGinn, D, Zackai, EH, et al Cardiovascular anomalies in patients diagnosed with a chromosome 22q11 deletion beyond 6 months of age. Pediatrics 2001;108,E104[CrossRef][Medline]
  13. McElhinney, DB, Clark, BJ, III, Weinberg, PM, et al Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol 2001;37,2114-2119[Abstract/Free Full Text]
  14. DelPizzo, A Problem of aortic vascular rings and other anomalies of the aortic arch. Br J Anaesth 1969;41,898-903[Abstract/Free Full Text]
  15. Bron, AO, Mensen, EA, Dijkman, JH, et al Dyspnoea persisting after surgery for a vascular ring. Eur Respir J 1994;7,2257-2259[Abstract]
  16. Bose, S, Hurst, TS, Cockcroft, DW Right-sided aortic arch presenting as refractory intraoperative and postoperative wheezing. Chest 1991;99,1308-1310[Abstract/Free Full Text]
  17. Bertrand, JM, Chartrand, C, Lamarre, A, et al Vascular ring: clinical and physiological assessment of pulmonary function following surgical correction. Pediatr Pulmonol 1986;2,378-383[ISI][Medline]
  18. Lowe, GM, Donaldson, JS, Backer, CL Vascular rings: 10-year review of imaging. Radiographics 1991;11,637-646[Abstract]
  19. Jung, JY, Almond, CH, Saab, SB, et al Surgical repair of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. J Thorac Cardiovasc Surg 1978;75,237-243[Abstract]
  20. Lam, CR, Kabbani, S, Arciniegas, E Symptomatic anomalies of the aortic arch. Surg Gynecol Obstet 1978;147,673-681[ISI][Medline]
  21. Bisset, GS, III, Strife, JL, Kirks, DR, et al Vascular rings: MR imaging. AJR Am J Roentgenol 1987;149,251-256[Abstract/Free Full Text]
  22. van Son, JA, Julsrud, PR, Hagler, DJ, et al Imaging strategies for vascular rings. Ann Thorac Surg 1994;57,604-610[Abstract]
  23. Dahlen, IB, Hillerdal, GN, Wegenius, GA, et al Breathlessness in a teenager. Eur Respir J 1993;6,145-147[ISI][Medline]
  24. Bevelaqua, F, Schicchi, JS, Haas, F, et al Aortic arch anomaly presenting as exercise-induced asthma. Am Rev Respir Dis 1989;140,805-808[ISI][Medline]




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