Ann Thorac Surg 2003;76:1730-1733
© 2003 The Society of Thoracic Surgeons
Case report
Hemoptysis from an unusual pulmonary arteriovenous malformation
Kin Hoi Thung, FRCSEda,
Alan D. L. Sihoe, MRCSa,
Innes Y. P. Wan, FRCSEda,
Tak Wai Lee, FRCSEda,
Randolph Wong, MBChBa,
Anthony P. C. Yim, MDa*
a Department of Surgery, Division of Cardiothoracic Surgery, Chinese University of Hong Kong, Shatin, Hong Kong, China
Accepted for publication April 3, 2003.
* Address reprint requests to Dr Yim, Division of Cardiothoracic Surgery, Department of Surgery, Chinese University of Hong Kong, Shatin, Hong Kong SAR, China
e-mail: yimap{at}cuhk.edu.hk
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Abstract
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We report the case of a 64-year-old woman who presented with massive hemoptysis. She was found to be bleeding from a pulmonary arteriovenous malformation in the right middle lobe, which had a peculiar blood supply from the right internal mammary artery. Video-assisted thoracic surgery lobectomy was successfully performed for this condition. Limitations of embolization as a treatment modality for this condition are discussed.
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Introduction
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Massive hemoptysis is a potentially life-threatening condition that requires prompt management. The most common cause is bronchiectasis, and pulmonary arteriovenous malformation remains one of the more unusual causes of this condition.
A 64-year-old woman with previously good health presented through the emergency room with a 1-day history of massive hemoptysis. She reported coughing up approximately 800 mL of fresh blood during a 24-hour period before arrival and suffered an episode of hypoxia immediately after admission. She was admitted to the intensive care unit and a chest radiograph showed right middle zone haziness (Fig 1).
Urgent fiber-optic bronchoscopy was performed and showed blood clots obstructing the right bronchus intermedius but no definite site of active bleeding was identified. Emergency bronchial angiography was performed to further localize the source of bleeding. An enhancing shadow highly suggestive of a pulmonary arteriovenous malformation was identified in the right middle lobe but the right bronchial arteries appeared normal. Instead, the right internal mammary artery (IMA) was noted to be abnormally thickened and there was rapid flow of contrast from the right IMA to the pulmonary arteriovenous malformation through feeding vessels. Blood from the pulmonary arteriovenous malformation was noted to drain to middle lobe pulmonary vein (Fig 2).
Selective embolization of the pulmonary arteriovenous malformation through the right IMA was considered but this was abandoned because the risk of systemic embolization was deemed by our interventional radiologists to be fairly high in this case. Furthermore, there is literature to suggest that where a pulmonary arteriovenous malformation is fed by a systemic vessel, treatment failure after embolization is higher compared with embolization for a bronchial artery-fed pulmonary arteriovenous malformation [1]. Noting that the patient was previously in good health with good exercise tolerance we therefore opted for definitive surgical management for her.
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Fig 2. Angiogram illustrating the right middle lobe pulmonary arteriovenous malformation with abnormal feeding vessels (arrow) from the right internal mammary artery.
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An urgent operation was arranged on the night of admission. A standard video-assisted thoracic surgery (VATS) approach for right middle lobectomy was used. Intraoperatively the right middle lobe was noted to be densely adherent to the chest wall. The feeding vessels from the right IMA to the right middle lobe were identified behind these adhesions and were individually ligated and divided. The right middle lobe parenchyma showed marked vascular congestion. An anatomical resection of the right middle lobe was performed using the standard VATS lobectomy approach, with the anterior port lengthened to a 4-cm utility minithoracotomy. Total operating time was 100 minutes and total blood loss was 150 mL.
The patient was extubated soon after surgery and transferred out of the intensive care unit to the general ward the next day. The patient made an uneventful recovery with no further hemoptysis and was discharged on the fourth postoperative day. Follow-up 6 months after surgery showed the patient remained well and free of symptoms.
Histopathologic examination of the resected right middle lobe revealed parenchymal hemorrhage and edema and focal collections of intraalveolar polymorphs. Dilated bronchi and bronchioles were apparent and contained blood clots and inflammatory exudates. Vessels in apposition to these ectatic bronchioles were also dilated with
irregular lumens and variable wall thicknesses. An increased number of irregular vessels were also noted at the peripheries of the lobe. The presence of pulmonary tumorlets, which consisted of clusters of neuroendocrine cells, further suggested of the diagnosis of pulmonary arteriovenous malformation (Figs 3 and 4)
[2].
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Fig 3. Surgical specimen illustrating ectactic bronchioles (white arrow) and dilated vessels (black arrow) near the visceral pleural surface.
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Fig 4. Histology illustrating bronchial epithelium with hemorrhages (narrow arrow), abnormally dilated and thickened wall vessels (white arrow), and island of tumorlets (thick arrow), which are suggestive of pulmonary arteriovenous malformation.
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Comment
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Pulmonary arteriovenous malformations are caused by abnormal connections between pulmonary arteries and pulmonary veins through a thin-walled aneurysm, which are most commonly congenital in nature although an acquired condition resulting from penetrating trauma has been described. They act as direct right-to-left shunts, resulting in dyspnea, fatigue, cyanosis, and polycythemia when the shunt is large. In addition because the pulmonary arteriovenous malformation bypasses the capillary bed the lung loses its filter function thus allowing emboli and bacteria to pass directly into the systemic circulation, resulting in stroke or cerebral abscess [3].
Since their first description at autopsy by Churton in 1897 various terms have been used to describe these abnormal communications including pulmonary arteriovenous fistulas, pulmonary arteriovenous aneurysms, hemangiomas of the lung, cavernous angiomas of the lung, pulmonary telangiectases, and pulmonary arteriovenous malformations. Pulmonary arteriovenous malformations exhibit a wide range of histology from diffuse telangiectasiae to large complex structures consisting of a bulbous aneurysmal sac between dilated feeding arteries and draining veins. Approximately 95% of these feeding arteries come from the pulmonary rather than systemic circulation [4]. Various systemic arterial supplies to pulmonary arteriovenous malformations were reported previously, most commonly from the bronchial artery, less so from the internal mammary artery [5] or even directly from the descending thoracic aorta [6].
The natural history of pulmonary arteriovenous malformations is such that they tend to increase in size, especially if multiple, and rarely regresse spontaneously. The mortality rate in historical reviews of untreated but symptomatic patients with pulmonary arteriovenous malformations over periods of 15 years ranges from 4% to 22% and in severe cases up to 40%. The abnormal vessels may bleed into a bronchus or the pleural cavity, sometimes with a fatal outcome [7].
A definitive diagnosis of a pulmonary arteriovenous malformation is made conventionally by angiography and this remains the gold standard allowing not only the location of the pathology but also mapping of the feeding vessels before surgical treatment or embolotherapy. However angiography is labor, cost, and radiation intensive and its use should be limited to individuals in whom noninvasive diagnostic tests strongly suggest the presence of a pulmonary arteriovenous malformation. Contrast and helical computed tomography scanning with three-dimensional reconstructions conveniently identifies small, multiple lesions; it can also identify thrombosed and, with the correct timing of contrast, recanalized structures. More recently the "ultrafast" three-dimensional magnetic resonance imaging angiograms have been touted as accurate but less invasive alternatives for characterizing pulmonary arteriovenous malformations. Intravenous digital subtraction angiography of pulmonary arteries is performed before formal catheter pulmonary angiography in some centers to visualize the pulmonary vasculature, particularly as part of an outpatient screening program. However this method is less likely to detect certain lesions than formal angiographic studies. Other noninvasive investigation including contrast echocardiography or radionuclide scanning of technetium-99m–labeled albumin microspheres or macroaggregates used to assess the presence of right-to-left shunting are under investigation.
As pulmonary arteriovenous malformations are direct, low-pressure, artery-to-vein connections that result in a direct right-to-left shunt the goals of treatment are threefold: (1) improve symptoms of dyspnea/hypoxemia; (2) prevent lung hemorrhage; and most important (3) prevent neurologic complications. Traditional options for the definitive management of pulmonary arteriovenous malformation presenting with massive hemoptysis include embolization and surgery. Embolization offers an effective therapy for patients who are poor surgical candidates or for patients whose lesions are too numerous to resect. Should adequate embolization of all feeding vessels be achieved, initial control of bleeding in the range of 80% to 100% has been reported. However even with such good initial control, embolization is associated with higher subsequent recurrent hemoptysis rates compared with surgery [1]. Surgical resection therefore remains the treatment of choice for patients who can tolerate the surgery [8].
The presence of a systemic arterial supply from the IMA to pulmonary arteriovenous malformation is a reminder that if one is confronted with hemoptysis and the blood supply is not identified on either bronchial artery or pulmonary artery injection, then IMA injection should always be considered.
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References
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- Novak K., Burger J., Urbanova S., et al. An AV aneurysm of a branch of the pulmonary artery associated with a pulmonary tumorlet. Rozhl Chir 2002;81:188-191.[Medline]
- Guttmacher A.E., Marchuk D.A., White R.I. Hereditary hemorrhagic telangiectasia. N Engl J Med 1995;333:918-924.[Free Full Text]
- Bosher L.H., Blake A., Byrd B.R. An analysis of the pathologic anatomy of pulmonary arteriovenous aneurysms with particular reference to the applicability of local excision. Surgery 1959;45:91-104.[Medline]
- Robinson L.A., Sabiston D.C., Jr Syndrome of congenital internal mammary-to-pulmonary arteriovenous fistula associated with mitral valve prolapse. Arch Surg 1981;116:1265-1273.[Abstract/Free Full Text]
- Kuwahara T., Ida M., Hamada T. A case of systemic arterial supply to the normal left basal segments with anomalous return to the left inferior pulmonary vein. Nihon Kyobu Shikkan Gakkai Sushi 1995;33:733-737.
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- Puskas J.D., Allen M.S., Moncure A.C., et al. Pulmonary arteriovenous malformations: therapeutic options. Ann Thorac Surg 1993;56:253-257.[Abstract]
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Abstract
Introduction
