Takayasu’s arteritis with bilateral pulmonary artery stenosis

Takayasu’s arteritis with bilateral pulmonary artery stenosis

J THORAC CARDIOVASC SURG 1987;94:246-50 Takayasu's arteritis with bilateral pulmonary artery stenosis Successful surgical correction The case of a ...

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J

THORAC CARDIOVASC SURG

1987;94:246-50

Takayasu's arteritis with bilateral pulmonary artery stenosis Successful surgical correction The case of a 60-year-old patient with severe stenosis of both proximal pulmonary arteries resulting from Takayasu's arteritis is reported. The thoracic aorta and pulmonary artery were calcified. Patch enlargement of the pulmonary arteries was successfully performed. Operative biopsies confirmed the diagnosis of nonspecific arteritis consistent with Takayasu's disease.

S. Chauvaud, M.D., L. Mace, M.D., P. Brunewald, M.D., J. L. Tricot, M.D., J. P. Camilleri, M.D., and A. Carpentier, M.D., Paris, France

Bmonary involvement is a widely established condition in Takayasu's arteritis. I, 2 Our case exhibites a granulomatous arteritis of the pulmonary artery and of the aorta consistent with Takayasu's disease. A few rare cases of pulmonary hypertension are associated with the disease, I but to our knowledge only one surgical case of pulmonary artery stenosis has been reported so far.' Case report A 60-year-old woman was referred to the Broussais Hospital for evaluation of a systolic murmur. She reported a history of pleural effusion of unknown origin in 1951. One year before her admission to our hospital she began having shortness of breath, cough, and recurrent episodes of hemoptysis and fever, up to a temperature of 38.5° C. On admission there was no cyanosis and the blood pressure was 130/80 mm Hg in both arms and legs. The heart rate was 90 beats/min. A Grade 3/6 systolic murmur was heard in the pulmonic area and a Grade 1/6 systolic murmur in the tricuspid area. There was no hepatomegaly. Pulmonary auscultation disclosed no abnormalities. The cardiothoracic ratio was 0,61 and pulmonary artery vascularization was diminished. The electrocardiogram revealed a normal rhythm with a QRS axis of +150 degrees, a right bundle branch block, and right ventricular hypertrophy. Echocardiography revealed dilatation of the right atrium and the right ventricle; the tricuspid and the mitral valves were

From the Departments of Cardio-vascular Surgery (Chauvaud, Mace, and Carpentier), Cardiology (Tricot), and Pathology (Brunewald and Camilleri), Hopital Broussais Paris, France. Received for publication June 19, 1986. Accepted for publication Aug. 5, 1986. Address for reprints: Sylvain Chauvaud, M.D., Department of Cardiovascular Surgery, Hopital Broussais, 96, rue Didot, 75674 Paris Cedex, France.

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Table I. Catheterization data Pressure (mm Hg) Preoperative RA RV PA LPA RPA LV Ao

9/0, 135/4, 120/4, 22/9, 20/8, 120/1, 120/68,

4 8 60

16 16

Postoperative 7/1, 4 60/1, 6 60/10, 30 30/10,19 20/10, T5

6 95

Legend: Preoperative catheterization disclosed a gradient of 110 nun Hg between the right ventricle and both pulmonary arteries. Six months after the surgical procedure,the gradient had fallen to 30 mm Hg on the leftside and 40 mm Hg on the right side. RA, Right atrium. RV, Right ventricle. PA, Main pulmonaryartery. LPA, Left pulmonaryartery. RPA, Right pulmonaryartery. LV, Left ventricle. Ao, Aorta.

normal and the ventricular septum was moderatly hypertrophied with normal motion. Bronchoscopyrevealed hypervascularization of the trachea. On fundus examination the usual arteriolar atherosclerotic lesions were found. Computed tomography showed calcification of the ascending and descending aorta, The main trunk of the pulmonary artery and its branches were calcified (Fig. I). The sedimentation rate was 91 mrn/I hour and 136 mm/z hours. Red cell count was 3,5 million/rnm', white cell count 12,600/mm 3, neutrophils 84%, and eosinophils0%. The hemoglobin level was 9.1 gm/IOO mI, hematocrit 30%, platelet count 400,000/mm 3, fibrinogen concentration 0.8 gm/IOO ml, serum protein level7.6 gm/IOO ml, and albumin concentration 3.66 gm/IOO ml. The tuberculin test was negative. At catheterization, pulmonary artery hypertension and a significant pressure gradient at the origin of the left and right pulmonary arteries were recorded (Table I). Intracardiac oxygen saturations were normal. The cardiac index was 2.75 L/m 2/min.

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August 1987

Fig.... Computed tomography of the thoracic aorta. Calcifications of the ascending (arrow) and descending aorta.

Fig. 2. Stenosis of the origin of the right pulmonary artery (A) and left pulmonary artery (B). Cineangiography demonstrated a severe stenosis of the origin of the left and right pulmonary arteries (Fig. 2). The right ventricular cavity was enlarged and was normally contractile. Mild tricuspid insufficiency was present. Digital angiography of the thoracic aorta disclosed only a slight narrowing of the carotid arteries and of the aortic isthmus. Surgical treatment was performed in May 1985. Exposure was obtained through a median sternotomy. Because the ascending aorta was calcified, cardiopulmonary bypass was instituted between the left femoral artery and the venae cavae. Myocardial protection was obtained by retrograde infusion of crystalloid cardioplegicsolution into the right atrium at a body temperature of 28° C. Inflamed tissues were found in close

contact with both the aorta and the main pulmonary artery. The ascending aorta was cross-clamped and transected anterior to the right pulmonary artery, 4 cm above the aortic anulus. The aortic wall was thickened and fibrotic with calcifications. The bifurcation of the pulmonary artery was opened transversely and the incision was extended to the left and right branches beyond the stenosis. The wall of the pulmonary artery was thickened (5 mm) and inflamed with areas of calcifications (Fig. 3). The distal pulmonary arteries were normal. A diamond-shaped glutaraldehyde-preserved autologous pericardial patch was used to enlarge the pulmonary artery bifurcation and the origin of both branches. The autologous pericardium was treated with 0.6% buffered glu-

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Fig. 3. Diagram of operative view. A, Stenosis of both pulmonary arteries. The internal diameter was 3 mm and the walls were thickened (5 mm). Exposure of the bifurcation was obtained by the transection of the ascending aorta. B, Enlargement of both pulmonary arteries and bifurcation with an autologous pericardial patch. operation (Table I), and pulmonary arteriography confirmed a mild residual stenosis on the left side (Fig. 4). During the operation, biopsyspecimens were taken from the pulmonary artery and the ascending aorta. The specimens were fixed in Bouin's solution and routinely processed for histologic study. Section were stained with hematoxylin and eosin, Masson's trichrome, and Weigert's elastic stains. The pulmonary trunk was the most severely involved. It was thickened up to 0.6 em with fibrosis completely replacing the arterial fibers and myocytes of the media and extending to the adventitia. Fibrosis contained inflammatory foci of mononuclear cells and rare giant cells (Fig. 5). The ascending aorta exhibited fibrous replacement limited to the outermost layers of the media and fibrous thickening of the adventitia. Inflammatory infiltrates were less prominent. In both.vessels, nonspecific fibrous thickening of intima was present. Fig. 4. Postoperative angiogram (6 months). A mild stenosis at the origin of the left pulmonary artery was present.

taraldehyde solution for 15 minutes and subsequently washed in saline for 5 minutes according to a technique developed by one of us (A.c.). Before the procedure, the internal diameter of the pulmonary artery branches was 3 mm. After the procedure, the internal diameter measured with a Hegar dilator was 13 mm. After cardiopulmonary bypass, the radial artery pressure was 140/70 mm Hg and the right ventricular pressure 60/2-7 mm Hg. The postoperative course was uneventful. Six months after the operation, a Grade 1/6 systolic murmur was present over the pulmonic area. Dyspnea disappeared. The patient was given continuous corticosteroid and antituberculosis therapy. Cardiac catheterization was performed 6 months after the

Discussion

Takayasu's arteritis is a nonspecific inflammatory arteritis affecting the aorta, its branches, and the pulmonary arteries. The incidence of pulmonary artery involvement is between 14%4 and 40% to 50%,I.2.5 according to various reports, This disease may cause aneurysms, occlusions, or stenoses of the systemic and pulmonary arteries. Instances of pulmonary localization are rare, but some common characteristics could be distinguished. The right pulmonary artery and its branches are most often involved."? Bilateral pulmonary arteritis is exceptional." 4, 6 Stenotic lesions of the pulmonary artery are most often found in the segmental and lobar branches. I. 5 A case of occlusion of the pulmonary

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Takayasu's arteritis

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Fig. 5. Histologic examination. A, Peripheraldestruction of elastic fibers in the media of the aorta. Thick fibrous adventitial ring with inflammatory cells (arrow). (Weigert's elastic and hematoxylin and eosin stain; original magnification x80.) B, Dense inflammatory infiltrate in the pulmonary artery wall with mononuclear cells and giant cells (arrow). (Hematoxylin and eosin stain; original magnification x80.) artery has been reported by Kosuka.' Aneurysmal dilatation of the right pulmonary has also been reported.2.7.8 Hypervascularization has been described in subsegmental arteries." Little data are available from the literature concerning the pathologic features of pulmonary arteritis in Takayasu's disease.'? Intimal fibrosis in small pulmonary arteries was described with plexiform or angiomatoid lesions. The usual surgical approach to Takayasu's arteritis is bypass graft of the stenotic lesions.11, 12 Only one case of surgical treatment of stenotic lesions of the pulmonary artery has been reported.' A patch angioplasty of the left branch combined with a tubular graft between the right and left pulmonary arteries was performed. Because of the risk of endoluminal fibrous proliferation in rightsided conduits," we preferred to avoid the use of a conduit. The transaortic approach to the bifurcation and the right pulmonary artery used for closure of the Waterston shunt 14, 15 proved to be useful. Despite the calcified tissues, the repair of the pulmonary artery and the aorta was achieved without difficulty. The choice of the patch material was based on laboratory studies and the extensive use without failure of this material in our institution in the past 5 years (Carpentier A. Unpub-

lished data). Glutaraldehyde preserves pericardial integrity and avoids long-term retraction or stretching. Pulmonary hypertension is responsible for the morbidity and the long-term mortality of Takayasu's arteritis." Despite the rarity of pulmonary artery involvement, surgical treatment of proximal pulmonary artery stenosis may significantly modify the course of the disease. REFERENCES 1. Lupi-Herrera E, Sanchez GI, Horowitz S, Gutierrez E.

2. 3.

4. 5.

Pulmonary artery involvement in Takayasu's arteritis. Chest 1975;67:69. Ishikawa K. Natural history and classification of occlusive thrombo-aortopathy. (Takayasu's disease). Circulation 1978;57:27. Moore JW, Reardib LJ, Cooley DA, Vargo TA. Severe Takayasu's arteritis of the pulmonaryarteries: report of a case with successful surgical treatment. J Am Coll Cardiol 1985;5:369. Lupi-Herrera E, SanchezGT, Marcushamer J, Mispireta J, HorwitzS, Vela JE. Takayasu's arteritis: clinical study of 107 cases. Am Heart J 1977;93:94. Kosuka T, NosakiT, Sato K, Imara K. Aortitissyndrome with special reference to pulmonary vascular changes. Acta Radiol 1968;7:25-31.

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6. Lande A, Gross A. Total aortography in the diagnosis of Takayasu's arteritis. A1R 1972;116:165. 7. Kawai C, Ishikawa K, Kato M, Ishii Y, Nakao K. "Pulmonary pulseless disease:" pulmonary involvement in so-called Takayasu's disease. Chest 1978;73:651. 8. Chauvaud S, Gillon lF, Valois M, Dubost C. Localisation pulmonaire atypique de la maladie de Takayasu. Ann Cardiol Angeiol 1983;32:55. 9. Berkmen YM, Lande A. Chest roentgenography as a window to the diagnosis of Takayasu's arteritis. A1R 1975;125:842.

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10. Rose AG, Halper 1, Factor SM. Primary arteriopathy in Takayasu's disease. Arch Pathol Lab Med 1984; 108:644. 11. Bloss RS, Duncan 1M, Cooley DA, Leatherman LL,

Schnee Ml. Takayasu's arteritis: surgical considerations. Ann Thorac Surg 1978;27:574. 12. Duncan 1M, Cooley DA. Surgical considerations in

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aortitis with special emphasis on Takayasu's arteritis. Texas Heart Inst 1 1983;10:233. Agarwal KC, Edwards WD, Feldt RH, Danielson GK, Puga Fl, McGoon DC. Clinicopathological correlates of obstructed right-sided porcine-valved extracardiac conduits. 1 THORAC CARDIOVASC SURG 1981;81:591. Ergin MA, Griepp RB. Total correction of tetralogy of Fallot: how to deal with the complicated ascending aorta-right pulmonary artery anastomosis. 1 THORAC CARDIOVASC SURG 1979;77:469. Yamamoto N, Reul Gl, Kidd lN, Cooley DA, Hallman GL. A new approach to repair of pulmonary branch stenosis following ascending aorta to right pulmonary artery anastomosis. Ann Thorac Surg 1976,21:237. Ishikawa K. Survival and morbidity after diagnosis of occlusive thromboaortopathy (Takayasu's disease). Am 1 Cardiol 19lN;47:1026.