Eur J Cardiothorac Surg 2006;30:405-407
© 2006 Elsevier Science NL
Pulmonary arteriovenous malformation in a patient with tuberculosis — an association?
Rajesh Thomas*,
Devasahayam J. Christopher,
Jacob Chacko,
Joyce Ponnaiya
Department of Pulmonary Medicine, Christian Medical College, Vellore, India
Received 16 March 2006;
accepted 22 April 2006.
* Corresponding author. Tel.: +91 416 2282859/511; fax: +91 416 2232035/103. (Email: rajesh{at}cmcvellore.ac.in; drrajeshthomas{at}rediffmail.com).
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Abstract
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We report a case of pulmonary arteriovenous malformation (PAVM) and associated pulmonary tuberculosis in a young girl who presented with hemoptysis. As intra-arterial coil embolization did not result in improvement of symptoms, surgical resection of the PAVM was performed, resulting in dramatic clinical improvement. Histopathology revealed a large PAVM, and in addition, caseous necrotizing granulomatous inflammation, suggestive of pulmonary tuberculosis. This case is being reported since the association of large PAVM and tuberculosis is very rare, and both could present with hemoptysis.
Abbreviations: PAVM = pulmonary arteriovenous malformation
Key Words: Pulmonary arteriovenous malformation (PAVM) • Pulmonary tuberculosis
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1. Introduction
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Pulmonary arteriovenous malformations (PAVM) are usually congenital; however, they may also occur in many acquired conditions. The association of AVM with pulmonary tuberculosis is very rare.
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2. Case summary
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A 14-year-old girl presented with two episodes of small-volume hemoptysis and syncope over a period of eight months, and breathlessness on exertion. There was no history of gastro-intestinal or mucosal bleed in the past. Initially she was evaluated at a local hospital and chest radiograph done then showed a small left hilar opacity. She was started on empirical anti-tuberculous treatment. However, subsequent chest radiographs revealed progressive increase in the size of the hilar opacity following which she was referred to us.
On examination, she had central cyanosis and grade 3 clubbing. General examination was otherwise unremarkable. There was no evidence of telangiectatic lesions on the nasal mucosa or skin. A continuous murmur, which increased on inspiration, was audible in the left mammary area on auscultation of the precordium.
Blood investigations revealed a hemoglobin level of 18 g%. Arterial blood gas analysis showed hypoxemia (PaO2 of 48.2 mmHg), which was not correctable despite oxygen supplementation. A large left perihilar shadow, which appeared vascular, was noted on the chest radiograph. A diagnosis of PAVM was considered based on the clinical and radiological findings. Spiral CT pulmonary angiogram (Fig. 1
) showed a large, predominantly pulmonary, arteriovenous malformation with right-to-left shunt in the left upper lobe and lingula. Echocardiogram showed no evidence of valvular heart disease. Pulmonary angiography revealed that the arterial supply was mainly from the left upper lobe pulmonary artery, with some supply from the left lower lobe artery and a large feeding vessel from the internal mammary artery. The venous drainage was into the left superior pulmonary vein. The rest of the lung appeared normal. Trans-catheter coil embolization was done following which there was no significant improvement in symptoms and oxygenation.
Subsequently, she developed a bout of massive hemoptysis. In view of the fact that the PAVM was localized to the left upper lobe, and since trans-catheter coil embolization had failed to control the symptoms, a left upper lobectomy was performed. Histopathology of the resected lung confirmed the presence of an arteriovenous malformation and in addition, evidence of necrotizing granulomatous inflammation with a focal area of caseous necrosis, consistent with tuberculosis (Fig. 2
). Following surgery, the underlying left lower lobe re-expanded, and the cyanosis and hypoxemia resolved. She was treated with the standard anti-tuberculous regime for six months. One year later, she remained completely symptom-free and with no new radiological lesions.
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Fig. 2. Granulomatous inflammation with focus of caseous necrosis (arrow) seen adjacent to pulmonary arteriovenous malformation.
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3. Discussion
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Pulmonary arteriovenous malformations were first described in 1897 and consist of abnormal communications between pulmonary veins and arteries [1]. Eighty to ninety percent of PAVMs are simple, with all feeding arteries arising from a single sub-segmental artery [2]. PAVM that is perfused by more than one sub-segmental artery, as in this case, is defined as complex.
PAVM of the lung are usually congenital in origin. Hereditary hemorrhagic telangiectasia (HHT, also called Rendu–Osler–Weber syndrome) is an autosomal dominant disorder believed to be the cause of up to 70% of all PAVM [3]. However, PAVM may occur in acquired conditions, particularly hepatic cirrhosis [4], and less commonly in schistosomiasis, mitral stenosis, trauma, actinomycosis, Fanconi's syndrome, and metastatic thyroid carcinoma [5,6]. In addition, systemic to pulmonary shunting could develop between bronchial arteries and pulmonary arteries, in chronic inflammatory conditions such as bronchiectasis [7].
PAVM related to Mycobacterium tuberculosis infection has only been infrequently reported [8,9]. Denlinger et al. [8] reported the case of a boy who developed a systemic-pulmonary AVM five years after being treated for a tuberculous empyema necessitans and pulmonary tuberculosis. The author had hypothesized that the PAVM formation with systemic collateralization was likely related to the inflammatory processes surrounding the tuberculous infection, which might have helped recruit local pulmonary and systemic arteries to feed the inflammatory mass. Builov [9] has also described a case of PAVM in association with pulmonary tuberculosis.
In the described case of PAVM, foci of caseous necrosis and necrotizing granulomatous inflammation, which are suggestive of pulmonary tuberculosis, were detected in the resected portion. However, a causal relationship between the tuberculous infection and PAVM cannot be readily evoked since the rest of the lung appeared normal on the CT scan with no evidence of other foci of infection. As the patient was from a region endemic for pulmonary tuberculosis, it is possible that the proximity of these disorders may have only been a coincidental occurrence.
However, it is also known that inflammatory processes surrounding a tubercular focus can help recruit local arteries. As there was no other cause or predisposing factor to explain the development of the PAVM and its progression, the possibility that the PAVM may have been a very unusual manifestation of localized tuberculous inflammation also merits consideration.
Successful management of a PAVM may involve surgery, selective embolization, or a combination of the two [10]. Surgical resection is indicated for larger PAVM or in patients who fail embolization. In our patient, surgical resection was successfully done after a failed embolization.
In summary, we report the association of a large PAVM and pulmonary tuberculosis in a patient who presented with hemoptysis, and its successful management. Combined intra-arterial embolization followed by thoracotomy with PAVM resection, and anti-tuberculous treatment resulted in the complete resolution of the symptoms.
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Acknowledgments
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The authors thank Dr Balamugesh Thangakunam, Department of Pulmonary Medicine, Christian Medical College, Vellore, India for his contribution to the management of the case and preparation of the manuscript.
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References
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- Denlinger CE, Egan TM, Jones DR. Acquired systemic-to-pulmonary arteriovenous malformation secondary to Mycobacterium tuberculosis empyema. Ann Thorac Surg 2002;74(4):1229-1231.[Abstract/Free Full Text]
- Builov VM. Case of arteriovenous aneurysm of the lungs in association with pulmonary tuberculosis. Probl Tuberk 1977;11:87.
- La Quaglia MP. Congenital anomalies. In: Pearson FG, Deslauriers J, Ginsberg RJ, Heibert CA, McKneally MF, Urschel HC, editors. Thoracic surgery. New York: Churchill Livingstone; 1995. pp. 411-432.
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Abstract
1. Introduction
