To the Editor:
We are presenting a patient who was admitted to our department because of an asthmatic attack and unexpectedly found to have a combination of congenital anomalies that we describe.
A 23-year-old man, smoker, with a long history of poorly controlled asthma, was admitted with acute exacerbation. A chest x-ray revealed a right-sided aortic arch as well as an azygous lobe on the right. A computed tomography (CT) of the chest confirmed the presence of the right aortic arch and the azygous lobe, but also revealed a right tracheal bronchus and an abnormal drainage of the right upper pulmonary vein (Fig. 1). A cardiac magnetic resonance image (MRI) revealed a partial anomalous connection of the vein (PAPVC) to the superior vena cava (Fig. 2). Bronchoscopy revealed a mild external compression of the right main bronchus (resulting from the presence of the right aortic arch) and the existence of a right tracheal bronchus (Fig. 3).
PAPVC is a rare congenital anomaly. In autopsy series, its incidence is estimated to 0.4% to 0.7% when the atrial septum is intact. When the anomaly is combined with an atrial septum defect, the incidence may reach 10%.1,2 Most of these patients remain asymptomatic during their lifetime. The most common evidence of the anomaly in children is an incidentally found cardiac murmur. In adults, dyspnea, palpitations, recurrent pulmonary infections, hemoptysis, chest pain, or peripheral edema are the main symptoms. A few cases of pulmonary edema have also been described.1,3,4 If only a single vein is anomalous, the physical examination may be entirely normal. Otherwise, signs of right-sided heart failure or pulmonary hypertension may be present.1 The findings on the chest radiography may be an abnormal left or right mediastinal border, cardiomegaly, or a “scimitar-like” shadow.5 The chest CT may reveal cardiomegaly, a right atrial or ventricular enlargement, and a pulmonary artery enlargement. The characteristic finding on MRI is the “broken ring sign” (Fig. 2, arrow). Contrast-enhanced 3-dimensional magnetic resonance angiography MRA is now a safe, rapid, noninvasive method to demonstrate these anomalous connections and estimate the pulmonary to systemic shunt.2,5-8
The right aortic arch is associated with a “vascular ring,” which may cause external bronchial or esophageal compression. In some patients, this condition may mimic a bronchial asthma.9-12 In our patient, the symptoms were attributed to bronchial asthma because he had a compatible history and his symptoms as well as his spirometry were improved after treatment.
A tracheal bronchus arises from the trachea or one of the main bronchi directed toward the upper lobes and its incidence is estimated at 0.1% to 2%. If a branch of the upper lobe is missing, then the tracheal bronchus is called a “displayed” one. When a normal anatomy is present, it is called “supernumentary.” In a case when it ends blindly, then the variation is called “diverticulum.” The majority of cases of tracheal bronchus are asymptomatic.13-15 Despite a multitude of anomalies, our patients had no direct symptoms.
Kokkonouzis Ioannis, MD
Psathakis Kostas, MD
Opsimoulis Panagiotis, MD
Mermigkis Charalampos, MD
Tsintiris Kostas, MD
Department of Pneumonology, Army General Hospital of Athens, Athens, Greece
1. Gustafson RA, Warden HE, Murray GF. Partial anomalous pulmonary venous connection of the right side of the heart. J Thorac Cardiovasc Surg
2. Whang ZJ, Reddy GP, Gotway MB, et al. Cardiovascular shunts: MR imaging evaluation. Radiographics
3. Van Meter C Jr, Leblanc JG, Culpper WS 3rd, et al. Partial anomalous pulmonary return. Circulation
4. Vanderheyden M, Goethals M, Van Hoe L. Partial anomalous pulmonary venous connection or scimitar syndrome. Heart
5. Haramati LB, Moche IE, Rivera VT, et al. Computed tomography of partial anomalous pulmonary connection in adults. J Comput Assist Tomogr
6. Tan RS, Behr ER, McKenna WJ, et al. Occult anomalous pulmonary venous drainage. The clinical value of cardiac magnetic resonance imaging. Circulation
7. Gareth MH, Roobottom C, Marshall A. Partial anomalous pulmonary venous drainage in adulthood. Clin Cardiol
8. Prasad SK, Soukias N, Hornung T. Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage. Circulation
9. Bron AO, Mensen EAM, Dijkman JH, et al. Dyspnoea persisting after surgery for a vascular ring. Eur Respir J
10. Panselinas E, Psathakis K, Kontogianni E, et al. Right aortic arch with a aberrant left artery causing tracheoesophageal compression. Journal of Bronchology
11. Katz M, Konen E, Rozenman J, et al. Spiral CT and 3D image reconstruction of vascular rings and associated tracheobronchial anomalies. J Comput Assist Tomogr
12. Kersting-Sommerhoff BA, Sechtem UP, Fisher MR, et al. MR imaging of congenital anomalies of the aortic arch. AJR Am J Roentgenol
13. Berrocal T, Madrid C, Novo S. Congenital anomalies of the tracheobronchial tree and mediastinum. Radiographics
14. Aoun YN, Velez E, Kenney L, et al. Tracheal bronchus. Respir Care
15. Moezzi HR, Sohrabpoor H, Fadaie A. A case of tracheal bronchus and congenital valve stenosis. Journal of Bronchology