Journal of Asthma, 47:946948, 2010 Copyright 2010 Informa Healthcare USA, Inc.

. ISSN: 0277-0903 print / 1532-4303 online DOI: 10.3109/02770903.2010.504877

CASE REPORT

An Unusual Cause of Dyspnea

SEVKET OZKAYA,
M . D .,1, B ILAL AND SERHAT

SENGUL, M.D.,2 SEMRA HAMSICI, FINDIK, M.D., F.C.C.P.3

M . D .,2

1 Department of Pulmonary Medicine, Rize University, Faculty of Medicine, Rize, Turkey Department of Pulmonary Medicine, Samsun Chest Diseases and Thoracic Surgery Hospital, Samsun, Turkey 3 Department of Pulmonary Medicine, Ondokuz Mayis University, Faculty of Medicine, Kurupelit, Samsun, Turkey 2

Background. Right-sided arcus aorta (RSAA) is a rare condition and usually asymptomatic. However, it may be symptomatic if it causes tracheal or esophageal compression. Methods. The authors evaluated clinical and radiological features of seven patients with RSAA who had the diagnosis between May 2006 and May 2009. Results. The authors found that the incidence of RSAA was 0.16% in patients who had applied to their clinic. The age of patients ranged from 17 to 55 years. The male to female ratio was 6/1. Four patients were symptomatic due to RSAA. Most common symptoms were dyspnea during exercise, which is similar to exercise-induced asthma and dysphagia. Two patients were misdiagnosed as asthma. The flow-volume curves on spirometry of the patients showed intrathoracic upper airway obstruction. Thorax magnetic resonance imaging (MRI) revealed marked narrowing of the tracheal air column due to external compression of RSAA in three patients. Conclusions. RSAA should be included in the differential diagnosis of asthma. Spirometry may help to suspect RSAA. Thorax computed tomography (CT) and/or MRI are the best imaging methods for the diagnosis of RSAA. Keywords: dyspnea; intrathoracic upper airway obstruction; right-sided arcus aorta; tracheal compression

INTRODUCTION The incidence of right-sided arcus aorta (RSAA) is about 0.10.2% in adults (1). This anomaly is usually asymptomatic. However, it may be symptomatic if it causes tracheal or esophageal compression. The most commonly reported symptoms were dyspnea and dysphagia (24). RSAA may mimic the bronchial asthma, especially during exercises. It often fails to be diagnosed due to the absence of symptoms and appropriate radiologic studies. We aimed to evaluate the clinical, radiological, and spirometric features in patients with right-sided arcus aorta. PATIENTS
AND

METHODS

A total of 41,490 patients were examined with chest radiography and RSAA was diagnosed in 7 patients at the 7th and 11th clinics of Samsun Chest Diseases and Thoracic Surgery Hospital between May 2006 and May 2009. The characteristics of patients including age, gender, symptoms, and radiological and spirometric findings were retrospectively evaluated and presented. Written consents of our patients and the approval of the institution were obtained to carry out our study. RESULTS The characteristics of the seven patients with RSAA are shown in Table 1. The mean age of the patients was

40.5 years (ranging from 17 to 55 years). The male to female ratio was 6:1. Four patients were symptomatic due to external compression of trachea and eosophagus by RSAA. The most common symptoms seen in these patients were exertional dyspnea and dysphagia. Two of these patients had asthma diagnosis because of their exertional dyspnea and received asthma treatment. They were taking long-acting -agonist (LABA) and inhaled corticosteroids, although there was no improvement in their symptoms. Other symptoms reported were related to lung cancer, chronic obstructive pulmonary disease (COPD), and nonspesific upper airway infection in three patients, respectively. Chest x-ray images of the patients revealed the absence of the shadow of arcus aorta on the left hemithorax (Figure 1). The flow-volume curves of exhalation throughout on spirometry showed an obstruction in the intrathoracic upper airways of two patients (Figure 2). Two patients had normal spirometric findings. Other spirometric findings were reported as restriction in two patients and obstruction in one patient. The diagnosis of RSAA was confirmed with thorax computed tomography (CT) and/or magnetic resonance imaging (MRI) in all patients (Figure 3). Also thorax MRI revealed marked narrowing of the tracheal air column due to external compression of RSAA in three patients (Figure 4AC).

DISCUSSION The right-sided arcus aorta (RSAA) is the most common anomaly of arcus aorta. The incidence of right-sided arcus aorta (RSAA) was reported as 0.10.2% in adults. This anomaly can be seen in both sexes (1). Assman 946

Corresponding author: Sevket Ozkaya, M.D., Assistant Professor, Department of Pulmonary Medicine, Rize University, Faculty of Medicine, Rize, Turkey; E-mail: ozkayasevket@yahoo.com

A RARE ANOMALY CONFUSED WITH ASTHMA

TABLE 1.Characteristics of patients Patients 1 2 3 4 5 6 7 Age/Gender 17 years/Male 55 years/Male 44 years/Male 54 years/Female 52 years/Male 35 years/Male 27 years/Male Symptoms Exertional dyspnea Cough, dyspnea Dyspnea, dysphagia Dyspnea, dysphagia Dyspnea Chest pain Exertional dyspnea Spirometry Intrathoracic upper airway obstruction Restriction Normal Restriction Obstruction Normal Intrathoracic upper airway obstruction Causes of symptoms

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Tracheal compression from RSAA Lung cancer Tracheal and esophageal compression from RSAA Tracheal and esophageal compression from RSAA and obesity COPD Nonspecific upper airway infection Tracheal compression from RSAA

obstruction in these patients. RSAA was confirmed by using thorax CT and/or MRI in all patients. Spirometry was normal in two patients. Spirometric findings were of

FIGURE 1.Chest radiography showing extended right upper mediastinal structures (white arrow).

first described the roentgen studies of this anomaly and Renander reported the first case in English literature (2). In Turkey, the incidence of RSAA was previously reported as 0.06% (6). In the present study, we found that the incidence of RSAA was 0.16%. RSAA is usually asymptomatic. But, it can be symptomatic due to tracheal and esophageal compression. Most common symptoms reported were dysphagia and dyspnea. Also, persistent cough and asthma-like symptoms may be seen due to tracheal compression (1, 5). Fndk et al. reported eight patients with right-sided arcus aorta. Four of them were symptomatic. Two of these patients suffered from exertional dyspnea, whereas the other two patients suffered from cough and dyspnea (6). In our study, four patients were symptomatic due to RSAA. According to published reports, some cases can be presented with dysphagia and dyspnea on exertion (7, 8). Some cases had the clinical diagnosis of exercise-induced asthma, as seen in our patients. Spirometry can be helpful in the diagnosis of suspected tracheal compression in symptomatic patients (9). The expirium loop of flow-volume curves on spirometry showed the plateau in two patients. According to these findings, we suspected intrathoracic upper airway

FIGURE 2.The expirium arm of the flow-volume curve on spirometry suggesting intrathoracic upper airway obstruction (black arrows).

FIGURE 3.Thorax CT image depicting external tracheal compression of right-sided arcus aorta (white arrows).

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S. OZKAYA ET AL. the restrictive type in two patients and obstructive type in one patient. Fndk et al. reported intrathoracic upper airway obstruction on spirometry in two patients and normal spirometry findings in five patients with RSAA. One of them was reported as restrictive (6). If there is no shadow of arcus aorta on the left side of chest radiography, RSAA should be suspected. Thorax CT and thorax MRI are the best methods for diagnosis. MRI should be the preferred diagnostic test because it is noninvasive and can clearly demarcate between intrathoracic vascular structures and the trachea. In this study, we demonstrated the external tracheal compression of RSAA with thorax MRI in two patients. To our knowledge, these are the first images presented in the literature. In conclusion, RSAA should be included in the differential diagnosis of asthma, especially in cases with intractable exertional dyspnea. Spirometry can help to suspect tracheal compression of RSAA. MRI seems to be the best diagnostic tool in symptomatic patients because of its capability to show the relationship between aorta and trachea in detail.

DECLARATION

OF I NTEREST

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. REFERENCES
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FIGURE 4.Coronal section of thoracic MR images showing marked narrowing on tracheal air column in three patients (white arrows).

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