Ann Thorac Surg 2009;88:281-283. doi:10.1016/j.athoracsur.2008.09.061
© 2009 The Society of Thoracic Surgeons
Case Reports
Idiopathic Pulmonary Vein Thrombosis: A Rare Cause of Massive Hemoptysis
Gerard R. Alexander, MBChB*,
Anu Reddi, FCS (Cardio),
Darshan Reddy, MBChB
Department of Cardiothoracic Surgery, Inkosi Albert Luthuli Central Hospital, University of KwaZulu-Natal, Durban, South Africa
Accepted for publication September 24, 2008.
* Address correspondence to Dr Alexander, Department of Cardiothoracic Surgery, Inkosi Albert Luthuli Central Hospital, PB X03, Mayville, Durban, 4058, South Africa (Email: gerardale{at}ialch.co.za).
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Abstract
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The case history of an adult female with massive hemoptysis due to idiopathic left inferior pulmonary vein thrombosis necessitating lower lobectomy is presented with a review of the current literature.
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Introduction
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Massive hemoptysis in our environment is almost always due to inflammatory lung disease (ie, active tuberculosis or bronchiectasis) and very rarely other causes. Irrespective of the cause, when the disease is localized to the lobe or lung in an otherwise suitable surgical candidacy, after attention to contributory factors, lung resection is the preferred modality of treatment, with few exceptions.
A 47-year-old African female was referred to our thoracic surgical unit with a history (3 days previously) of massive hemoptysis associated with left chest pain and mild dyspnea. There was no surgical history noted. Clinical examination was noncontributory, except for digital clubbing, decreased air entry, and crackles in the left base posteriorly. The chest roentgenogram (Fig 1) showed homogenous opacification of the left lower lobe with concomitant high-resolution computed tomographic scan (HRCT) features of air-space consolidation of the same lobe (Fig 2). Laboratory investigation revealed anemia with a hemoglobin level of 8.3 g/dL, a leukocytosis of 30.07 x 109/L, an ESR of 91, albumin of 33 g/L, a normal urea, electrolytes, and coagulation profile. She was deemed suitable for management by lung resection and a left lower lobectomy was undertaken with relative urgency. Thoracotomy was immediately preceded by bronchoscopy, which confirmed our suspicion of bleeding from the left lower lobe. No other pathology was noted endoscopically. At operation the lobe was consolidated and congested. The inferior pulmonary vein was anatomically normal in site and size, and except for the acute thrombus internally, the vessel was structurally normal. In addition, there was no other obvious pathology (eg, inflammatory fibrosis) in close proximity to the vessel, whereas division of the pulmonary artery and bronchus was unremarkable. Macroscopic cut-sections of the lung parenchyma demonstrated red hepatization with thrombosis of the pulmonary venous system. The postoperative course was uneventful. On histology of the resected lobe, features of a recent hemorrhagic infarction were seen. There was no evidence of tumor in the pulmonary veins. In view of the operative and microscopic findings, an echocardiogram was undertaken that showed a normal left atrium with no evidence of a thrombus. In addition, the mitral valve leaflet tips were mildly thickened with normal mobility, the left ventricular contractility was good, and the pulmonary systolic pressure was 31 mm Hg. At the time of this writing, a work-up for an underlying pro-thrombotic state was ongoing.
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Fig 2. Preoperative computed tomographic scan demonstrating air-space consolidation of the left lower lobe.
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Comment
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Based on our scouring of published articles, spontaneous or idiopathic pulmonary vein thrombosis (PVT), as a cause of massive hemoptysis necessitating lung resection is unrecorded in the Anglophone scientific literature. As the entity is relatively unknown and inadequately described in traditional texts, the review that follows attempts to address some pertinent areas of interest (the notation "spontaneous" or "idiopathic" is prefixed to PVT in this case, as no precipitating cause could be identified).
The PVT has been documented after pulmonary resection [1], lung transplantation [2], and radiofrequency ablation for atrial fibrillation [3]. Direct extension of the tumor into proximal pulmonary venules with subsequent stasis and thrombosis of larger distal veins may mimic primary PVT [4].
The pathophysiology of PVT is analogous to that of mitral stenosis [5, 6]. A significant decrease in the mitral valve area causes an increase in the left atrioventricular gradient and pulmonary venous pressure with compensatory pulmonary arteriolar vasoconstriction. "Leaky capillaries," interstitial edema, and increased uptake by lymphatics through the mechanism of Starling forces occur. Elevated pulmonary artery pressure, right ventricular end-diastolic pressure, and right ventricular dilatation may ultimately result.
In animal studies, Wyatt and colleagues [5] described the sequential changes in the lung after ligation of the pulmonary veins (ie, congestion, edema, extravasation of serum, and blood in the alveoli resulting in total consolidation).
Hurwitz and colleagues [6] documented the development of post-alveolar bronchial venous channels after ligation of the pulmonary veins. Oxygen saturation in the azygos veins was noted to be similar to that of systemic arterial oxygen content (right-to-right shunt). Histology at 3 to 6 months revealed complete resolution of the consolidation, possibly due to re-canalization of the thrombosed pulmonary vein. Other compensatory mechanisms also seen include the development of dense vascular adhesions between lung and the chest wall [6].
The clinical presentation of PVT may be nonspecific, with symptoms such as dyspnea, cough, hemoptysis, pleuritic chest pain, and radiographic features of consolidation of the lobe or lung [1]. After lobectomy, thrombosis commonly occurs early in the postoperative period [1]. In lung transplantation, 15% occurs within 48 hours postoperatively, with features of pulmonary edema, pulmonary infection, or infarction making distinction from acute rejection or reperfusion injury a vexation [2].
Peripheral embolization to cerebral, coronary, or limb circulations from fragmentation of thrombus within the pulmonary veins may result in well-recognized clinical features of organ ischemia. The source of the emboli may however produce a clinical dilemma [7].
The diagnosis of PVT, as noted in previous publications, is almost always tentative and established retrospectively after lung resection or autopsy. However, it may be suspected from a radiographic series that shows abrupt and complete opacification of residual lung after lobectomy or bi-lobectomy [1]. On computed tomographic scan of the thorax, a longer delay after contrast administration (pulmonary venous phase) is necessary for adequate visualization of the pulmonary veins and cardiac chambers [8]. Transesophageal echocardiography may demonstrate thrombus when it extends into the large distal veins and left atrium [8].
Little is known about the definitive treatment of PVT. Thrombosis associated with lobectomy may be treated by thrombectomy. Anticoagulation used judiciously (in the absence of hemorrhage) may prevent clot propagation and embolization [1]. Regression of lung changes and functional improvement have been described with this form of therapy [6].
Lobectomy or completion pneumonectomy is indicated for clinical deterioration or if lung gangrene supervenes [1].
Expectant observation for lack of a diagnosis or treatment strategy may resignedly be obligatory in some [6, 8].
There are few publications on the subject of PVT. Although causal factors have been identified, the diagnosis may be elusive and treatment remains enigmatic. Although PVT as a cause of massive hemoptysis is exceptionally rare, the entity must nevertheless be considered, especially in circumstances in which there is a predisposition for such obvious exclusionary common conditions.
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Acknowledgments
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The authors would like to thank the Department of Health, KwaZulu-Natal, and the Administration at Inkosi Albert Luthuli Central Hospital, especially Dr Joshua's kind permission to publish this article.
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References
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- Hovaguimian H, Morris JF, Gately HL, Floten HS. Pulmonary vein thrombosis following bilobectomy Chest 1991;99:1515-1516.[Abstract/Free Full Text]
- Sarsam MA, Yonan NA, Beton D, McMaster D, Deiraniya AK. Early pulmonary vein thrombosis after single lung transplantation J Heart Lung Transplant 1993;12:17-19.[Medline]
- Yataco J, Stoller JK. Pulmonary venous thrombosis and infarction complicating pulmonary venous stenosis following radiofrequency ablation Respiratory Care 2004;49(12):1525-1527.[Medline]
- Nelson E, Klein JS. Pulmonary infarction resulting from metastatic osteogenic sarcoma with pulmonary venous tumour thrombus AJR 2000;174:531-533.[Free Full Text]
- Wyatt PJ, Burke RD, Hanlon RC. Morphologic study of canine lungs after ligation of the pulmonary veins Am J Pathol 1953;29:291-303.[Medline]
- Hurwitz A, Calabresi M, Cooke WR, Liebow AA. An experimental study of the venous collateral circulation of the lung: functional observations J Thorac Surg 1954;28:241-246.[Medline]
- Rodriguez L, Vandervoort P, Garcia MJ. Pulmonary vein thrombosis with peripheral embolization Chest 1996;109:846-847.[Abstract/Free Full Text]
- Selvidge SDD, Gavant ML. Idiopathic pulmonary vein thrombosis: Detection by CT and MR Imaging AJR 1999;172:1639-1641.[Free Full Text]
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Abstract
Introduction
