Surgical Case of Isolated Pulmonary Takayasu's Arteritis

Abstract

akayasu's arteritis is a primary, chronic, progressive, vascular inflammatory disease that causes stenosis and/or aneurysmal dilatation of the aorta and aortic arch branches, and in 50-80% of cases the pulmonary arteries as well. 1 However, isolated pulmonary Takayasu's arteritis is very uncommon. We present a case of isolated pulmonary Takayasu's arteritis and describe the successful surgical treatment of this rare condition. Case ReportA 67-year-old woman with a 6-month history of progressive exertional dyspnea was referred in January 2003. Laboratory examination showed an erythrocyte sedimentation rate (ESR) of 36 mm at 1 h and 70 mm at 2 h; C-reactive protein, 0.8 mg/dl; white blood cells, 9,800 / l; platelets, 42×10 4 / l. Rheumatoid factor, antinuclear antibody and Wasserman reaction were all negative. Human leucocyte antigens (HLA) were B52, A24 and DR2.Pulmonary perfusion scintigraphy revealed complete absence of perfusate in the left lung and no defects in the right lung. Magnetic resonance imaging revealed remarkable wall thickness of the pulmonary trunk and proximal right pulmonary artery ( Fig 1A). Magnetic resonance angiography (MRA) demonstrated severe stenosis of the right main pulmonary artery and obstruction of the left main pulmonary artery (Fig 1B). The aortic arch and its primary branches were normal. Cardiac catheterization showed that the systolic pressure of the main pulmonary artery was 85 mmHg and 25 mmHg in the right pulmonary artery, and the arterial pressure gradient was 60 mmHg. There was no stenosis of the coronary arteries and no incompetence of the aortic valves. A diagnosis of Takayasu's arteritis based on the angiographic findings was confirmed by the laboratory, although the inflammatory response was mild.In March 2003, the patient underwent surgical treatment. Exposure was obtained through a median sternotomy and total cardiopulmonary bypass, involving cannulation of the superior and inferior vena cava through the right atrium and of the ascending aorta, was instituted. Inflamed tissues were found in close contact with both the aorta and right pulmonary artery. Under cardioplegic cardiac arrest, the main trunk and bifurcation of the pulmonary artery were Circ J 2005; 69: 500 -502 (Received February 16, 2004; revised manuscript received May 21, 2004; accepted June 2, 2004 CASE REPORTS

4D-flow MRI assessment of blood flow before and after endovascular intervention in a patient with pulmonary hypertension due to isolated pulmonary artery involvement in large vessel vasculitis

“…Pulmonary artery involvement in Takayasu's arteritis has been reported in approximately 50%-80% of patients [11]. Isolated involvement of the pulmonary artery is rarely reported [12], [13], [14], [15], [16], [17]. Patients typically show PH and right heart symptoms, due to pulmonary involvement.…”

Isolated large vessel pulmonary vasculitis leading to pulmonary artery aneurysm formation: a case report and literature review

“…In two previous reports of isolated pulmonary Takayasu’s arteritis, satisfactory outcomes were obtained after surgical repair without other therapeutic interventions. 21 , 22 In other cases, either high dose immunosuppression or surgical repair followed by immunosuppression was pursued. 17 – 19 , 23 In our patient, the decision to proceed with surgery was mainly due to the severity and rapid extension of the PAA.…”

Isolated pulmonary vasculitis: Case report and literature review

“…Some of them are considered isolated forms of Takayasu's arteritis [9,11,12] or giant cell arteritis [13], while others are pulmonary giant cell vasculitis related to malignancies [8]. However, only a few cases of isolated large-vessel pulmonary vasculitis presenting as a SOV have been published in adults [14,15] and children [16].…”