Takayasu’s Arteritis: Surgical Considerations Robert S. Bloss, M.D., J. Michael Duncan, M.D., Denton A. Cooley, M.D., Louis L. Leatherman, M.D., a n d Mark J. Schnee, M.D.

ABSTRACT Takayasu’s arteritis, the nonspecific occlusive disease originally believed to affect young Oriental women exclusively, was identified in a 19year-old man who had severe involvement of the aortic arch and its branches. Successful surgical treatment was accomplished with a bypass from the ascending aorta to the supraceliac abdominal aorta using a tube graft. The right vertebral artery, as the single patent vessel reaching the head, was revascularized distal to the stenosis with a tube graft that extended from the aortic graft. Revascularization of ischemic organ systems and body areas may be accomplished in most patients with Takayasu’s arteritis using the bypass concept and fabric conduit grafts.

Texas Heart Institute typifies these considerations.

A 19-year-old carpenter was hospitalized after two episodes of spontaneous, severe left chest pain that radiated to the left arm and lasted 15 minutes before completely subsiding. He also complained of dizziness when gazing upward. When he was 2 years old, he had been hospitalized for two and one-half months because of a series of severe skin lesions which became necrotic, slowly spread from the left arm, and eventually involved the entire body except the head. Despite high fever, wound cultures were negative for pyogenic bacterial growth. Several courses of treatment with antibiotics were not helpful. The patient gradually improved, and Takayasu’s arteritis is a nonspecific occlusive disease that can affect the aortic arch, descend- the wounds healed with residual diffuse cutaing thoracic and abdominal aorta, the major neous scarring. Two months after the illness, a aortic branches, and even the pulmonary artery murmur was heard in the left upper chest. [5-9, 111. The many names attributed to varia- Further evaluation was refused by the parents tions of this disease, i.e., pulseless disease [161, at that time. The patient then had a normal aortic arch syndrome [14], atypical coarctation childhood and adolescence, except for fatigue of [6], and Martorell’s syndrome [lo], underscore the upper extremities on heavy exertion, until the lack of both a unifying pathogenetic mech- the present symptoms occurred. Physical examination at this admission reanism and strict diagnostic criteria. The disease was described by Savory [15] in vealed a thin man who was 180 cm tall and 1856 but came to be associated with Takayasu weighed 59 kg. Blood pressure was 110/70 mm [171 who, in 1908, discussed the ocular changes Hg in both arms and 180/80 mm Hg in both and symptoms with which it is often asso- legs. Diffuse scarring over the entire body ciated. Although Takayasu’s arteritis was origi- below the head had a peculiar latticework apnally believed to affect young Oriental women pearance with raised 1-mm ridges. No pulses only, numerous cases have been reported dur- were palpable in the neck. Pulses in the upper ing the last two decades involving men as well extremities were notably diminished, but those as women outside the Orient [l, 7, 91. Certain in the lower extremities were normal. Physical characteristics of the vessel involvement pre- examination of the chest revealed mild carsent a challenge in the surgical management of diomegaly, with a grade 3/6 systolic murmur the disease. A young man treated recently at the heard best in the left upper chest. The results of neurological and ocular examination were norFrom the Divisions of Surgery and Cardiology of the Texas mal. An electrocardiogram revealed evidence of Heart Institute of St. Luke’s Episcopal and Texas Children’s left ventricular hypertrophy, and a roentgenoHospitals, Houston, TX. gram of the chest demonstrated prominent Accepted for publication Sept 1, 1978. notching of the ribs. Cardiac catheterization Address reprint requests to Dr. Cooley, Texas Heart Instidisclosed normal coronary arteries. An arch tute, P.O. Box 20345, Houston, TX 77025. 574 0003-4975/79/060574-06$01.25 @ 1978 by Robert S. Bloss

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aorta, a 16-mm woven low-porosity Dacron tube graft* was anastomosed to the ascending aorta. The graft was then passed to the right of the right atrium, ventral to the inferior vena aortogram demonstrated total occlusion of both cava, and through a T-shaped opening made in common carotid and subclavian arteries at their the fibrous portion of the diaphragm. A parorigins (Fig 1). A large right vertebral artery, tially occluding clamp was placed on the previwith 60% proximal stenosis, led off from the ously exposed supraceliac portion of the abdiffusely stenotic innominate artery and was dominal aorta, and the distal graft was sutured the only patent vessel reaching the head. The in place [19). Revascularization of the right intracranial vessels were filled through the cirvertebral artery was achieved by anastomosing cle of Willis from the vertebral artery. The aorta an 8-mm woven Dacron tube graft* proximally was stenotic in a tapered manner at the level of to the aortic graft (parallel to the course of the the aortic arch, and there were prominent interinnominate artery) and to the right vertebral costal collaterals. Results of abdominal aortic artery, distal to its stenotic portion. and selective renal arteriograms were normal. No neurological problems developed despite Operation was performed through a median occlusion of the right vertebral artery for 15 sternotomy that extended superiorly into the minutes while the anastomosis was being comright neck and inferiorly to the umbilicus (Fig pleted. The patient's recovery was uneventful, 2). Dissection revealed evidence of previous and follow-up angiography revealed patent arteritis with fibrosis and no palpable pulses in grafts (Fig 3). the great vessels except in the innominate artery leading to the right vertebral artery. There were no palpable plaques' With a partially *Meadox Medicals, Inc., P.O. Box 5307, Oakland, NJ occluding atraumatic clamp on the ascending 07436.

Fig 1 . Preoperative angiogram and line interpretation showing the extent of vascular occlusion in a patient with Takayasu's arteritis.

576 The Annals of Thoracic Surgery Vol 27 No 6 June 1979

sternotomy extended right neck

Fig 2 . The surgical approach. Note the single vascular slcpply to the brain through the right vertebral artery.

Fig 3. Postoperative angiogram and line interpretation showing effective bypass grafting from the ascending aorta to the abdominal aorta and right vertebral artery.

577 Case Report: Bloss et al: Takayasu’s Arteritis

Type II 30%

!i? .A

Type IV

(?up

to 45%)

.-

Fig 4 . Types of vascular involvement in Takayasu‘s arteritis.

Comment The different nomenclature given the nonspecific arteritis of Savory and Takayasu is confusing. Judge and co-workers 171 recommended restricting the name to “Takayasu’s arteritis” until the cause of the disease is firmly established. Three types of vascular involvement were described by Edmunds [5] (Fig 4).In type I, disease is confined to the aortic arch and its branches (50% of patients). In type 11, the entire aorta and its large branches are involved (30%). In type 111, disease is limited to the distal thoracoabdominal aorta (10 to 15%). Lupi and colleagues [81 added a fourth type which may involve any of the features of the other types in addition to the pulmonary artery. Careful evaluation of pulmonary angiograms may reveal this fourth type to include as many as 45% of patients [91. Although the etiology of the disease remains unclear, tuberculosis [91, autoimmune factors [ll], rheumatic diseases [l], and syphilis 1121 have been implicated. Histologically, the entire thickness of the vascular wall is involved in a severe, chronic inflammatory process. Two-thirds of the patients with the disease experience an acute phase characterized mainly by systemic and cardiovascular symptoms. The

patient is usually between 11and 30 years old at onset of the disease, but it has been described in younger children and elderly people 15, 7, 9, 181. Several hundred cases have been reported, with a female predominance (8.5:l) in all series. Symptoms that often herald the onset of the disease include fever, malaise, anorexia, arthritis or arthralgia, dyspnea, and headache [9, 111. A history of previous tuberculous infections is reported by 50% of the patients [9]. Various skin manifestations, including erythema multiforme, erythema nodosum, or Bazin’s erythema induratum, may occur early in the course of the disease [l, 7,91. Severe skin lesions and fever were the early manifestations in our patient. The acute phase often is accompanied or followed by symptoms of ischemia. Evidence of cerebral and ocular impairment, such as dizziness, syncope and decreased visual acuity, with or without cataracts, may supervene. More than 90% of the patients have palpable pulse deficits and vascular bruits at various anatomical sites [91. Hypertension is common, especially when the renal arteries are involved, and may occur only in the lower extremities. The term rezlerse coarctation has been used to describe this condition. Early fatigue of the masticatory and upper extremity musculature may develop. Most patients have electrocardiographic abnormalities and left ventricular hypertrophy,

578 The Annals of Thoracic Surgery Vol 27 No 6 June 1979

and some complain of severe angina [9]. Rib notching may be evident on chest roentgenograms, and superficial collateral vessels visible on the trunk [l,5, 91. The clinical course of the disease is one of gradual deterioration. Because of the early age and insidiousness of onset and the gradual development of extensive collaterals, occlusive disease is often advanced when the patient consults a physician. The vertebral arteries can be the only supply of blood to the head, which demonstrates the special importance of an intact circle of Willis in these patients [ll. Pulses often are notably diminished in the upper extremities and prominent in the lower. Abdominal bruits may be heard. The lethal or crippling sequelae of this disease have been emphasized [I, 111. Although corticosteroids reportedly have been helpful in treatment of patients with acute manifestations of the disease, there is little evidence in favor of their use in patients with the chronic form [l, 91. At the time of surgical evaluation, patients usually exhibit hypertension or ischemia resulting from the chronic nature of the disease. Operation for treatment of renal artery involvement in patients with hypertension often is unsuccessful. Renal artery bypass grafts commonly occlude; and in one series, even nephrectomy was ineffective in 50% of patients who had unilateral renal artery lesions [91. Fortunately, most patients with hypertension caused by Takayasu’s arteritis respond well to medical management. Bypass grafting should be performed in patients with occlusive disease of the aortic arch and its branches. Thromboendarterectomy usually is unsuccessful because of the nonatherosclerotic, fibrotic nature of the disease [21. The proximal nature of the disease process and lack of distal involvement, however, allow bypass grafting of the involved vessels. Several patients were reported who may have had Takayasu’s arteritis and were treated with bypass grafts from the ascending aorta [4]. The grafts reportedly showed no tendency toward occlusion. According to Austen and Shaw [21, a knitted Dacron graft from the ascending aorta to both common carotid arteries remained patent

for three years in a 28-year-old Chinese woman. Riehl and Brown [13], however, reported postoperative thrombosis of an initially successful graft. The patency of the grafts in our patient serves to support this technique as a useful surgical approach in treating patients with this enigmatic disease. We have found that a bypass from the ascending aorta to the abdominal aorta can be helpful in patients with complex obstructions of the aortic arch [19]. We have also used the supraceliac abdominal aorta as a bypass site in patients with left ventricular outflow tract obstruction [3]. The graft, which includes a prosthetic valve, is placed from the left ventricular apex to the abdominal aorta above the celiac axis. In our patient the ascending aorta-to-abdominal aortic graft proved a useful conduit -for bypassing occlusive disease of the aortic arch. In addition, the high-flow, low-porosity graft was readily accessible as a proximal anastomotic site for the bypass to the right vertebral artery. Although this single patent artery to the brain was totally occluded for 15 minutes, no neurological deficits resulted. Apparently, extensive collaterals continued to supply the brain during clamping, and there may also have been previous cerebral adaptation to the chronic hypoperfused state. The proximal nature of vascular involvement and the availability of satisfactory prosthetic and autogenous grafts have set the stage for imaginative and successful revascularization. We believe aggressive surgical therapy using the bypass principle is indicated in patients with advanced stages of Takayasu’s arteritis.

References 1. Ask-Upmark E: O n the ”pulseless disease” out-

side of Japan. Acta Med Scand 169:467, 1961 2. Austen GW, Shaw RS: Surgical treatment of pulseless (Takayasu’s) disease. N Engl J Med 270:1228, 1964 3. Cooley DA, Norman JC, Mullins CE, et al: Left ventricle to abdominal aorta conduit for relief of aortic stenosis. Cardiovasc Dis Bull Texas Heart Inst 2:376, 1975 4. Crawford ES, DeBakey ME, Morris GC Jr, et al: Thrombo-obliterative disease of the great vessels arising from the aortic arch. J Thorac Cardiovasc Surg 43:38, 1962

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5. Edmunds LH Jr: Trauma and occlusive disease, study and critical review of twenty-one autopsy cases reported in Japan. Angiography 14:225, in Collateral Circulation in Clinical Surgery. 1962 Edited by DE Strandness Jr. Philadelphia, Saun13. Riehl JL, Brown WH: Takayasu’s arteritis: an ders, 1969, pp 270-286 autoimmune disease. Arch Neurol 12:92, 1965 6. Inada K, Shimizu H, Yokoyama T: Pulseless disease and atypical coarctation of the aorta with 14. Ross RS, McKusick VA: Aortic arch syndrome: special reference to their genesis. Surgery 52:433, diminished or absent pulses arising from the 1962 arch of the aorta. Arch Intern Med 92:701, 1953 7. Judge RD, Currier RD, Gracie WA, et al: 15. Savory WS: Case of a young woman in whom the main arteries of both upper extremities and of Takayasu‘s arteritis and the aortic arch syndrome. Am J Med 32:379, 1962 the left side of the neck were throughout completely obliterated. Med Chir Trans (Lond) 39: 8. Lupi HE, Sanchez TG, Horwitz S, et al: Pulmo205, 1856 nary artery involvement in Takayasu’s arteritis. 16. Shimizu K, Sano K: Pulseless disease. J NeuroChest 67:69, 1975 9. Lupi-Herrera E, Sanchez-Torres G, Marcushamer pathol Clin Neurol 1:37, 1951 J, et al: Takayasu’s arteritis: clinical study of 107 17. Takayasu M: Case with unusual changes of the central vessels in the retina. Acta SOCOpthalmol cases. Am Heart J 93:94, 1977 Jpn 12:554, 1908 10. Martorell-Otzet F, Fabre Tersol J: El sindrome de obliteracion d e 10s troncos supraaorticos. Med 18. M‘arshaw JB, Spach MS: Takayasu’s disease (primary aortitis) in childhood. Pediatrics 35:620, Clin (Barcelona) 2:26, 1944 1965 11. Nakao K, Ikida M, Kimata S, et al: Takayasu’s arteritis: clinical report of eighty-four cases and 19. Wukasch DC, Cooley DA, Sandiford FM, et al: immunologic studies of seven cases. Circulation Ascending aorta-abdominal aorta bypass: indications, technique, and report of 12 patients. 35:1141, 1967 Ann Thorac Surg 23:442, 1977 12. Nasu T: Pathology of pulseless disease: systemic

Takayasu's arteritis: surgical considerations.

Takayasu’s Arteritis: Surgical Considerations Robert S. Bloss, M.D., J. Michael Duncan, M.D., Denton A. Cooley, M.D., Louis L. Leatherman, M.D., a n d...
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