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A 52-Year-Old Man With Palpitations and a Solitary Pulmonary NoduleMan With Palpitations and Pulmonary Nodule FREE TO VIEW

Arooj S. Kayani, MD; Richard D. Sue, MD
Author and Funding Information

From the Department of Internal Medicine (Dr Kayani), St. Joseph’s Hospital and Medical Center; and Arizona Pulmonology Specialists Ltd (Dr Sue), Phoenix, AZ.

CORRESPONDENCE TO: Arooj S. Kayani, MD, Department of Internal Medicine, St. Joseph’s Hospital and Medical Center, 500 W Thomas Rd, Ste 900, Phoenix AZ 85013; e-mail: aroojkayani@live.com


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2015;147(6):e220-e223. doi:10.1378/chest.14-2148
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A 52-year-old white man presented to a pulmonary clinic for evaluation of a 2.3 × 1.7 cm lung nodule. The patient had originally presented to his cardiologist for palpitations. The palpitations were described as a “fluttering” sensation, occurring daily, more often at rest, but not associated with syncope. At the time, he denied dyspnea, paroxysmal nocturnal dyspnea, or orthopnea. The patient had a coronary artery calcium scoring test done, which revealed a lobulated, well-circumscribed, smoothly marginated lower lobe nodule, and he was sent to a pulmonary clinic for further evaluation. The patient denied shortness of breath, chest pain, cough, wheezing, or hemoptysis. He denied fatigue, night sweats, or weight loss. He had a 1 pack-year smoking history and stopped cigarettes 30 years ago but still smoked two to three cigars monthly. His family history was only significant for early coronary artery disease. He was an avid marathon runner who worked as an athletic equipment manager for a prominent sports team in Arizona.

Figures in this Article

Vital signs included temperature of 36.1°C; BP, 120/77 mm Hg; heart rate, 47 beats/min; and respiratory rate, 12 breaths/min. The rest of the physical examination was unremarkable. The patient’s lungs were clear to auscultation bilaterally.

A complete blood count and basic metabolic panel were normal. Coccidioidomycosis serology was negative. Exercise stress testing, echocardiogram, and Holter monitor testing were normal. CT scan of the chest with contrast showed an approximately 2.3 × 1.7 cm lobulated and well-circumscribed vascular structure within the right lower lobe, associated with surrounding bronchiectatic changes (Fig 1). Arterial feeding vessels appeared to cross the major fissure.

Figure Jump LinkFigure 1 –  A, B, Pulmonary nodule within the right lower lobe with surrounding bronchiectatic changes, as seen on the initial CT scan with contrast.Grahic Jump Location
What diagnosis can explain the imaging findings?
Diagnosis: Pulmonary varices

A pulmonary vein varix is defined by the presence of abnormal dilatation of a segment of the pulmonary vein either as an isolated finding or in association with cardiovascular disease. It is extremely rare. Pulmonary varices have been reported in all age groups, with equal frequency in men and women. The cause of pulmonary varices is unclear. Pulmonary varices are generally divided into congenital and acquired types. The congenital type may develop from an anomalous formation of capillaries during embryogenesis, even though histologically no intrinsic defect in the structure of the vessel wall has been found. The acquired type may result from pulmonary venous hypertension or portal hypertension, which can both lead to mediastinal collateral circulation. Pulmonary venous hypertension can be caused by mitral valve disease, pulmonary venous occlusive disease, coarctation of the aorta, and the Fontan procedure, among other causes. Regression of pulmonary varices after valve replacement has been reported, which suggests that pulmonary venous hypertension plays an important role in the development of pulmonary varices. Portal hypertension resulting in pulmonary varices can be either hepatic or extrahepatic. Bronchial varices have been reported subsequent to sclerotherapy for esophageal varices.

Pulmonary varices may be classified into three morphologic types: saccular, tortuous, or confluent. Saccular type varices are localized, oval or saccular dilatations of the pulmonary vein. Tortuous type varices are twisted, elongated dilatations of the pulmonary vein, whereas confluent type varices are dilatations in the confluence of the pulmonary vein. Most of the confluent and tortuous types of varices have been seen in the right lower lobe. Saccular varices have been seen in both left upper lobe and right lower lobe. Sixty-two percent of confluent varices and 19% of tortuous varices were found to be associated with heart disease, in particular mitral valvular disease.

Pulmonary varices are usually asymptomatic. They are most commonly found in the right lower lobe, left upper lobe, and right upper lobe. Some patients may present with hemoptysis. Chest pain, cough, and rare presentations like dysphagia and middle lobe syndrome have also been described.

Pulmonary varix appears on a chest radiograph as a round lobulated nodule with well-defined margins. Contrast-enhanced CT scan and MRI are of limited diagnostic value and may show the pulmonary varix as an intensified nodule or distension of the pulmonary vein. Differential diagnosis includes conditions like lung tumor, lymphadenopathy, granulomatous disease, infection, and pulmonary arteriovenous malformation (AVM). Multislice helical CT scan with or without three-dimensional reconstruction may suggest the diagnosis of pulmonary varix based on the demonstration of an enlarged vein draining into the left atrium without any arterial abnormality, but even this modality may not allow to differentiate pulmonary varix from AVM, especially in cases where the pulmonary varices are originating from the peripheral lung zone. Transthoracic echocardiography has a limited role in identifying pulmonary varices, although transesophageal echocardiography can identify pulmonary varices close to the heart. Bronchoscopy may reveal varices located in the membranous part of trachea and bronchi. The mainstay for diagnosis is pulmonary angiography. Pulmonary angiography can verify the diagnosis on the basis of the following criteria proposed by Batram and Strickland: (1) normal arterial phase, (2) filling of the varix in the venous phase, (3) direct draining into the left atrium, (4) delayed emptying compared with the other pulmonary veins, and (5) localization to the proximal portion of the vein. In rare cases, diagnostic thoracotomy has been performed, when diagnosis was unclear by other modalities.

Pulmonary varices are usually benign in their clinical course; therefore, direct intervention is generally not required. Embolization or surgical resection is needed for pulmonary varices complicated by hemoptysis or thrombosis. Invasive therapy can be considered for patients with large varices, but no selection criteria exist to identify high-risk patients for preventative interventions. Pulmonary varices associated with mitral valvular disease may regress after valvular repair or replacement. In patients with portal hypertension, pulmonary varices complicated by life-threatening hemoptysis may compel aggressive management. In such cases, transjugular portosystemic intrahepatic shunt or portosystemic shunting by laparotomy may be therapeutic options. Although rare, serious complications can occur. Recurrent infections, spontaneous rupture of the varix into the pleural cavity or a bronchus causing fatal hemorrhage, and cerebral embolism secondary to thrombus in the variceal lumen have all been reported.

Clinical Course

The patient was first believed to have an AVM based on the abnormally enhanced vascular structure on CT scan of the chest. A pulmonary angiogram was performed next. Arterial phase of pulmonary angiogram showed communication between the lower lobe segmental pulmonary artery branch and a large venous structure (Fig 2). Embolization was performed of what was mistakenly considered to be an AVM. Postdeployment right pulmonary angiogram shows delayed but still visible opacification of the venous structure in the right lower lung field, which confirmed the diagnosis of pulmonary varix (Fig 3). Upon review of the pulmonary angiogram again, it was noted that the right superior pulmonary vein was truncated and did not empty into the left atrium (Fig 4) but emptied into the right inferior venous varix instead (Fig 5). In the light of these findings, and absence of portal as well as pulmonary venous hypertension, the pulmonary varix was believed to be of congenital origin. Because the patient was asymptomatic, it was decided to observe the pulmonary varix. The patient complained of mild pleuritic chest pain after the procedure, which resolved after a few days. His palpitations resolved spontaneously, and no cause was identified.

Figure Jump LinkFigure 2 –  Arterial phase of pulmonary angiogram showing communication between the lower lobe segmental pulmonary artery branch and a large venous structure.Grahic Jump Location
Figure Jump LinkFigure 3 –  Post deployment right pulmonary angiogram shows very delayed but still visible opacification of the venous structure in the right lower lung field.Grahic Jump Location
Figure Jump LinkFigure 4 –  Right superior pulmonary vein is truncated and does not empty into the left atrium vs the left side, which emptied normally.Grahic Jump Location
Figure Jump LinkFigure 5 –  Superior segment of the right lung emptying into the right inferior venous varix.Grahic Jump Location

  • 1. Pulmonary vein varix is a rare anomaly characterized by abnormal dilatation of a segment of the pulmonary vein.

  • 2. Pulmonary varix can either be congenital or acquired. Acquired cases may result from pulmonary venous hypertension or portal hypertension.

  • 3. Most pulmonary varices are asymptomatic and are found incidentally on chest imaging.

  • 4. Pulmonary angiography is necessary to confirm the diagnosis and differentiate a pulmonary varix from AVM.

  • 5. Treatment is indicated only for pulmonary varices complicated by hemoptysis or thrombosis.

Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Other contributions: CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Batram C, Strickland B. Pulmonary varices. Br J Radiol. 1971;44(528):927-935.
 
Uyama T, Monden Y, Harada K, et al. Pulmonary varices: a case report and review of the literature. Jpn J Surg. 1988;18(3):359-362.
 
Mannes GP, van der Jagt EJ, Postmus PE. An asymptomatic hilar mass. Chest. 1992;101(2):553-554.
 
Shiraishi J, Tatsumi T, Kimata M, et al. Echocardiographic diagnosis of pulmonary vein varix. Circ J. 2003;67(9):796-798.
 
Nagahiro I, Toda D, Andou A, Shimizu N. A case of bronchial varices due to extrahepatic portal hypertension. Respiration. 2007;74(4):460-461.
 
Zhang X, Han HL, Shi ZL. Thoracic exploration for the diagnosis of multiple pulmonary vein varices: case report. Chin Med J (Engl). 2007;120(22):2053-2055.
 
Kumazoe H, Komori M, Ochiai R, Egashira R, Nakazono T, Kudo S. Pulmonary varix mimicking arteriovenous malformation. Clin Imaging. 2008;32(1):61-64.
 
Berecova Z, Neuschl V, Boruta P, Masura J, Ghersin E. A complex pulmonary vein varix-diagnosis with ECG gated MDCT, MRI and invasive pulmonary angiography. J Radiol Case Rep. 2012;6(12):9-16.
 
Nagatomo T, Saraya T, Masuda Y, et al. Two cases of bilateral bronchial artery varices: one with and one without bilateral coronary-to-pulmonary artery fistulas. Review and characterization of the clinical features of bronchial artery varices reported in Japan. Clin Radiol. 2012;67(12):1212-1217.
 

Figures

Figure Jump LinkFigure 1 –  A, B, Pulmonary nodule within the right lower lobe with surrounding bronchiectatic changes, as seen on the initial CT scan with contrast.Grahic Jump Location
Figure Jump LinkFigure 2 –  Arterial phase of pulmonary angiogram showing communication between the lower lobe segmental pulmonary artery branch and a large venous structure.Grahic Jump Location
Figure Jump LinkFigure 3 –  Post deployment right pulmonary angiogram shows very delayed but still visible opacification of the venous structure in the right lower lung field.Grahic Jump Location
Figure Jump LinkFigure 4 –  Right superior pulmonary vein is truncated and does not empty into the left atrium vs the left side, which emptied normally.Grahic Jump Location
Figure Jump LinkFigure 5 –  Superior segment of the right lung emptying into the right inferior venous varix.Grahic Jump Location

Tables

Suggested Readings

Batram C, Strickland B. Pulmonary varices. Br J Radiol. 1971;44(528):927-935.
 
Uyama T, Monden Y, Harada K, et al. Pulmonary varices: a case report and review of the literature. Jpn J Surg. 1988;18(3):359-362.
 
Mannes GP, van der Jagt EJ, Postmus PE. An asymptomatic hilar mass. Chest. 1992;101(2):553-554.
 
Shiraishi J, Tatsumi T, Kimata M, et al. Echocardiographic diagnosis of pulmonary vein varix. Circ J. 2003;67(9):796-798.
 
Nagahiro I, Toda D, Andou A, Shimizu N. A case of bronchial varices due to extrahepatic portal hypertension. Respiration. 2007;74(4):460-461.
 
Zhang X, Han HL, Shi ZL. Thoracic exploration for the diagnosis of multiple pulmonary vein varices: case report. Chin Med J (Engl). 2007;120(22):2053-2055.
 
Kumazoe H, Komori M, Ochiai R, Egashira R, Nakazono T, Kudo S. Pulmonary varix mimicking arteriovenous malformation. Clin Imaging. 2008;32(1):61-64.
 
Berecova Z, Neuschl V, Boruta P, Masura J, Ghersin E. A complex pulmonary vein varix-diagnosis with ECG gated MDCT, MRI and invasive pulmonary angiography. J Radiol Case Rep. 2012;6(12):9-16.
 
Nagatomo T, Saraya T, Masuda Y, et al. Two cases of bilateral bronchial artery varices: one with and one without bilateral coronary-to-pulmonary artery fistulas. Review and characterization of the clinical features of bronchial artery varices reported in Japan. Clin Radiol. 2012;67(12):1212-1217.
 
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