Ann Thorac Surg 2002;73:1977-1979
© 2002 The Society of Thoracic Surgeons
Case report
Pulmonary thromboendarterectomy in a patient with giant cell arteritis
Stephanie J. Brister, MDa*,
Kristine Wilson-Yang, PhDb,
Francesca V. Lobo, MDb,
Homer Yang, MDb,
Richard Skala, MDb
a Department of Surgery, University of Toronto, Toronto Canada
b Department of Anesthesia and Pathology, McMaster University, Hamilton, Ontario, Canada
Accepted for publication December 5, 2001.
* Address reprint requests to Dr Brister, University Health Network, General Division 14EN-214, 200 Elizabeth St, Toronto, Ontario MSG 2C4, Canada
e-mail: stephanie.brister{at}uhn.on.ca
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Abstract
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This report describes the case of a young woman presenting with signs and symptoms of chronic thromboembolic pulmonary hypertension who underwent pulmonary thromboendarterectomy (PTE) with concomitant coronary artery bypass. She died in the intensive care unit 1 day postoperatively. At autopsy the patient was found to have giant cell arteritis of the pulmonary arteries and ascending aorta. It is important to differentiate this disease from chronic thromboembolic pulmonary hypertension because its management and that of systemic vasculitis differs considerably.
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Introduction
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Approximately 300,000 pulmonary embolic events with mortality as great as 30% occur in the United States every year. Most emboli resolve with restoration of normal pulmonary hemodynamics. However some patients develop pulmonary hypertension secondary to chronic thromboembolic disease. The true incidence and underlying cause of the disease are unknown [1]. Other pulmonary diseases, eg, fibrosing mediastinitis [2] or pulmonary fibrous histiocytoma [3], may mimic chronic thromboembolic pulmonary hypertension. Infrequently, systemic vasculitis can result in pulmonary thrombosis. Kerr and colleagues [4] found Takayasu’s arteritis involving the pulmonary arteries in 4 patients who had been referred with the presumed diagnosis of chronic thromboembolic pulmonary hypertension. Giant cell arteritis of the pulmonary arteries has been rarely confirmed histologically. Ladanyi and Fraser [5] and Landrin and colleagues [6] reported single cases of elderly women with pulmonary artery thrombosis and giant cell arteritis diagnosed at autopsy and by temporal artery biopsy, respectively. Both articles review relevant literature.
A 28-year-old woman suffering from severe dyspnea was referred to the surgical service. Two years earlier, this previously healthy young woman had presented to her family physician with uveitis and was treated with eye drops. Over the next 11 months she gradually became breathless on exertion and presented with chest discomfort. Chest roentgenogram showed a right pleural effusion and she was treated for pneumonia. After 3 months chest discomfort recurred. Lung scan showed a high probability of pulmonary embolism, which was confirmed by angiogram. There was no laboratory evidence of deep venous thrombosis. Repeat lung scan after 3 months showed no resolution of the thrombus, and over the following year her dyspnea worsened.
While under the care of the surgical service, a workup for thrombotic disorders (antithrombin III, protein C and S, lupus anticoagulant) was conducted and found to be negative. Repeat ventilation perfusion scan suggested pulmonary embolism in the lower and lingular lobes of the left lung, and in the lateral segment of the right middle lobe. Pulmonary angiography confirmed a proximal thrombus in the left pulmonary artery with no vascularity in the lower two-thirds of the left lung (Fig 1).
Vascular flow was decreased to the right midlung (Fig 1). Pulmonary function tests showed a reduced forced vital capacity and forced expiratory volume in 1 second, which was 72% of normal, consistent with a restrictive process. Forced expiratory volume in 1 second fell 5% with exercise. Echocardiogram showed normal right and left ventricular size and function. During an exercise stress test, the patient reached stage 1 of the Bruce protocol. Electrocardiographic changes suggested myocardial ischemia of the right ventricle. The patient was referred for coronary angiography.
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Fig 1. Pulmonary angiogram demonstrating minimal vascularity in the lower two-thirds of the left lung with a proximal thrombotic occlusion. Decreased flow to the right middle and lower lobes is also seen (arrow).
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Coronary angiography showed a mildly dilated aortic root and more than 50% stenosis of the right (Fig 2)
and left main coronary arteries (Fig 3).
Pulmonary vascular resistance was elevated at rest (272 dynes/second/cm2) and increased (400 dynes/second/cm2) with exercise. After review of the clinical symptoms and laboratory results, the diagnosis of chronic thromboembolic pulmonary hypertension and coronary artery disease was made. Pulmonary thromboendarterectomy (PTE) and coronary artery bypass grafting were scheduled.
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Fig 2. Coronary angiogram demonstrating ostial stenosis of the right coronary artery (arrow). The remainder of the vessel is free of significant stenosis.
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Fig 3. Coronary angiogram demonstrating ostial stenosis of the left main coronary artery (arrow). The remainder of the vessel is free of significant stenosis.
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On admission, the patient had a respiratory tract infection. The operation was delayed until a course of antibiotics was completed. She had mild ataxia. A computed tomographic scan of the head was normal.
An operation was performed 12 days after admission. Anesthesia was induced with midazolam, sufentanil, rocuronium, and sevoflurane. Maintenance of midazolam, sufentanil, pancuronium, and vecuronium was administered. After aortic and venous cannulations, cardiopulmonary bypass was instituted, cooling to 20°C. The saphenous vein graft to the obtuse marginal was performed first, followed by an uneventful thromboendarterectomy of the right and left pulmonary arteries during two 20-minute periods of circulatory arrest. During rewarming, right and left internal thoracic arteries were grafted to the right coronary and left anterior descending arteries, respectively. Separation from cardiopulmonary bypass was unsuccessful. Right ventricular failure, secondary to a compromised right internal thoracic artery graft, was suspected. She was placed on total bypass and a new saphenous vein graft was placed to the right coronary artery.
With an intraaortic balloon pump and inotropic infusions including dopamine, epinephrine, nitroglycerine, amrinone, and norepinephrine, the patient was weaned from bypass after four unsuccessful attempts (cross clamp time: 285 minutes; cardiopulmonary bypass time: 452 minutes; circulatory arrest: 40 minutes). During the following 24 hours, the patient had progressive cardiac dysfunction and respiratory insufficiency. She expired the following day.
Microscopic examination of the endarterectomy specimen showed myointimal hyperplasia with variable luminal stenosis and segmental occlusive old organized thrombus. Final pathology at autopsy was compatible with granulomatous arteritis of the ascending aorta and pulmonary arteries (Figs 4 and 5).
Both coronary ostia were severely stenosed. The affected vessels showed extensive inflammation and medial destruction, with a lymphoplasmacellular infiltrate, multinucleated giant cells, granulomata, and areas of fibrovascular proliferation. The intima exhibited hyperplasia but no significant inflammation, whereas the adventitia showed patchy perivascular lymphocytic infiltrates and some fibrosis. Stains for acid fast bacilli and fungi were negative.
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Fig 4. Active medial inflammation in the pulmonary artery, with lymphocytes, plasma cells, giant cells, and fibrovascular proliferation. (Hematoxylin and eosin, x200.)
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Fig 5. Granulomatous inflammation in vascular media with prominent giant cells, right coronary ostium. (Hematoxylin and eosin, x400.)
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Comment
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This case demonstrates the difficulty in identifying patients with rare diseases of the pulmonary artery. The patient had a clinical history with physical symptoms and laboratory information compatible with chronic thromboembolic pulmonary hypertension. Her previous iritis and the significant ostial coronary artery disease, which presented on angiography, are also rare in young women. They may have indicated a more systemic disease.
Based on pathologic findings, the differential diagnosis includes giant cell arteritis and Takayasu’s disease. In its early stages, Takayasu’s disease may demonstrate a granulomatous panarteritis morphologically indistinguishable from the large vessel involvement of giant cell arteritis [7]. Histologically, in view of the relative prominence of giant cells and lack of significant adventitial and medial fibrosis, giant cell arteritis was favored.
Operation is the recommended treatment for chronic thromboembolic pulmonary hypertension, and increasing numbers of patients are being referred. Not all of these patients will have this disease. In this case, if the diagnosis of giant cell or Takayasu’s arteritis had been made preoperatively, a course of prednisone and cyclophosphamide would have been considered first. Symptomatic and clinical improvements have been reported with drug therapy [4, 6].
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Acknowledgments
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The authors wish to recognize the assistance of Angela Pelletier, CPC, and Diane Batt, CPC, perfusionists, Hamilton Health Sciences, Hamilton, Ontario, Canada.
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References
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Moser K.M. Venous thromboembolism. State of the art. Am Rev Respir Dis 1990;141:235-249.[Medline]
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Berry D.F., Buccigroni J., Peabody K.L., et al. Pulmonary vascular occlusion and fibrosing mediastinitis. Chest 1986;89:296-301.[Abstract/Free Full Text]
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Carlin B.W., Moser K.M. Pulmonary artery obstruction due to malignant fibrous histocytoma. Chest 1987;92:173-175.[Abstract/Free Full Text]
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Kerr K.M., Auger W.R., Fedullo P.F., et al. Large vessel pulmonary arteritis mimicking chronic thromboembolic disease. Am J Respir Crit Care Med 1995;52:367-373.
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Ladanyi M., Fraser R.S. Pulmonary involvement in giant cell arteritis. Arch Pathol Lab Med 1987;111:1178-1180.[Medline]
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Landrin I., Chassagne P., Bouanicke M., et al. Pulmonary artery thrombosis in giant cell arteritis. Ann Med Interne 1997;148(4):315-316.[Medline]
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Lie J.T. Diagnostic histopathology of major systemic and pulmonary vasculitic syndromes. Rheum Dis Clin North Am 1990;16(2):269-292.[Medline]
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Abstract
Introduction
