181 Ann Thorac Surg 45: , Feb Copyright by The Society of Thoracic Surgeons

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1 The Surgical Treatment of Aortic Regurgitation Secondary to Aortitis Tadashi Isomura, M.D., Kouichi Hisatomi, M.D., Izumi Yanagi, M.D., Syoujirou Shimada, M.D., Kenichi Uraguchi, M.D., Shigeaki Aoyagi, M.D., Kenichi Kosuga, M.D., and Kiroku Ohishi, M.D. ABSTRACT We describe the operative and perioperative management of 11 patients with aortic regurgitation due to aortitis. All patients required aortic valve replacement because of severely uncoapted cusps secondary to dilatation of the ascending aorta. The right coronary ostium was narrowed in 5 patients and consequently necessitated a smaller coronary tip for the administration of cardioplegic solution. To implant the prosthetic valve, pledgeted 2- Tevdek sutures were placed through the aortic valve annulus either from the ventricular side or from outside the aortic wall. Steroids were administered to 4 patients preoperatively and 8 patients postoperatively. Postoperative dehiscence of the prosthesis was seen in 1 of the 3 patients not given any steroids. We conclude that it is important to arrest the inflammatory reaction before operation and if the aortic valve must be replaced, to reinforce the implanted prosthesis with pledgeted sutures. Also, we suggest the possible importance of steroid therapy. Takayasu s disease can affect various arteries arising from the aorta, and is manifested clinically as pulseless disease. In Japan, most of the patients are young women. Typically the absence of a radial pulse and ophthalmologic symptoms are noted. Less frequently seen is involvement of the aortic valve because of dilatation of the ascending aorta. This symptom is present when the aortic wall is affected by inflammation. We report here our experience with aortic regurgitation caused by idiopathic aortitis involving the ascending aorta. Material and Methods During the period May, 198, to June, 1985, we performed aortic valve replacement in 18 patients. Eleven of them had severe aortic regurgitation due to idiopathic aortitis. The clinical symptoms were either congestive heart failure or angina. Blood pressure was measured in both arms. Creatinine clearance and blood urea nitrogen were measured to assess renal function. The serum level of C-reactive protein (CRP) and the erythrocyte sedimenta- From the Second Department of Surgery, Kurume University Hospital, Kurume, Fukuoka, Japan. Accepted for publication Sept 1, 1987 Address reprint requests to Dr. Isomura, The Second Department of Surgery, Kurume University Hospital, 67 Asahi-Machi, Kurume, Fukuoka, Japan 83. tion rate (ESR) were examined to allow comparison of the inflammatory reaction before and after operation. Aortography was performed before operation to clarify the degree of aortic regurgitation and to define the extent of the aortitis. Coronary angiography was done preoperatively in 9 patients. Intraoperatively, biopsy specimens were obtained from the ascending aortic wall for histological study. The specimens were fixed in 2% buffered formalin and examined morphologically. Steroid therapy was administered to the majority of patients preoperatively, postoperatively, or both. Results Clinical Features Symptoms of congestive heart failure were seen in all of the patients except Patients 4 and 8 (Table 1). In Patient 4, mild fever of unknown origin was noted in In 1968, she was found to have an absent pulse in the left arm as well as a heart murmur. There were no major symptoms until 1975, when angina at rest suddenly developed. The patient continued to have moderate symptoms of angina, which was associated with hypertension, and was admitted to the hospital for further examination in During the latest admission, a severe ventricular arrhythmia occurred suddenly, and an emergency operation was performed. Symptoms of chest discomfort and of angina on exertion or at rest were present in all patients except Patients 2 and 9. Patients 5, 6, 7, and 8 had only mild chest discomfort. In Patient 1, angina on exertion developed when she was 28 years old, and she experienced angina several times a day, including at rest at the age of 34 years. By the time of operation, she had angina at rest more than ten times a day, even though she was on a regimen of medical treatment. At operation, in addition to the findings of a thickened, inflamed aortic wall and uncoapted aortic valve leaflets, the right coronary ostium was almost completely occluded by intimal hypertrophy. Hypertension was present in 6 patients, 2 of whom had borderline hypertension. Peripheral vessel lesions were seen in 5 patients. Angiograms revealed occlusion of the left subclavian artery in 2 patients and stenosis of the right axillary artery in 2. In addition, in Patient 11, the left subclavian artery was completely occluded and the right axillary artery was stenotic. The peripheral right pulmonary arteries were also occluded. Preoperative cardiac catheterization was performed in 9 patients, and the aortogram showed grade 314 or Ann Thorac Surg 45: , Feb Copyright 1988 by The Society of Thoracic Surgeons

2 182 The Annals of Thoracic Surgery Vol 45 No 2 February 1988 Table 1. Clinical Features in 11 Patients with Aortic Regurgitation Patient No., Age (yr), Sex 1. 39, F 2. 45, M 3. 52, F 4. 48, F 5. 6, F M 7. 59, F 8. 45, F 9. 41, F 1. 53, F , F Initial Symptoms Heart murmur Palpitation Mild fever Chest discomfort, dyspnea Palpitation on exertion, dyspnea, and edema Cardiomegaly Hypertension Palpitation, pulseless left arm "Angina score: 1 + = mild; 2 + = moderate; 3 + = severe. Congestive Duration after Heart Initial Symptoms Angina" Failure 9 Y' 3 mo 12 mo 15 yr 22 mo 21 mo 1 yr 2 Y' 11 yr 2 Y' 3 Y' r t h o p n e a r t h o p n e a Edema aortic regurgitation. Mild mitral regurgitation was seen in 4 patients. Renal dysfunction was noted in 5 patients: it was moderate in 3 and mild in 1. Patient 7 was considered to have severe renal dysfunction. Urine output was 4 to 9 ml per day, and the creatinine clearance was 15.2 mv min. Operative Findings and Treatment Our usual technique of cardiopulmonary bypass with aortic cannulation through the ascending aorta and dual venous cannulation through the right atrium was used in 6 patients. In the remaining 5 patients, the aortic cannula was placed in either the femoral artery or the external iliac artery because the dilated, thick-walled, inflamed aorta was markedly adherent to the surrounding tissues. Crystalloid cardioplegic solution was administered directly through both coronary orifices after the aortic root was opened. Smaller coronary tips were necessary for infusion of the solution in 4 patients because of coronary ostial narrowing by extension of the aortic inflammation to the ostia. In only Patient 1, the right coronary ostium was severely stenotic because of thickened intimal tissue at the ostium. The thickened tissue was then resected, and the ostium was reconstructed with running 6- Prolene suture. In all patients, the aortic valve leaflets were normal but severely uncoapted because of a thickened, dilated aortic annulus (Fig l), and the aortic valve was replaced with a Bjork-Shiley prosthetic valve. The leaflets were excised, and pledgeted 2- Tevdek sutures were placed through the annulus either from the ventricular side or from outside the aortic wall (Fig 2). The annulus and the aortic wall were thick but very fragile, and careful attention was paid in placing the sutures. The thick, fragile, inflamed aortic wall was dissected during the opening of the aortic root in Patient 8 (Fig 3), and was reapprox- Fig 1, Typical gross finding of severely uncoapted aortic cusps due to annular dilatation. imated with 5- Prolene. In all patients, the aortotomy was closed with a two-layer technique: the inner layer was a running horizontal mattress suture of 4- Prolene and the outer layer, an over-and-over whip stitch with 4- Prolene. Preoperative steroid therapy was initiated in 4 pa-

3 183 Isomura et al: Aortitis with Aortic Regurgitation Pledgetted 2- Tevdek Sutures - - c Left Ventricl Fig 2. Aortic valve replacement. Stitches are placed either from outside the aortic wall or from the left ventricular side. A B Fig 3. (Patient 8.) (A) The ascending aorta (Ao, arrows) is grossly inflamed and dilated. (B) The aortic wall is fragile and dissected (arrows) in the medial layer because of inflammation and destroyed elastic fibers. (H6E; x5 before 25% reduction.)

4 184 The Annals of Thoracic Surgery Vol 45 No 2 February 1988 Table 2. Steroid Therapy and Pathological Findings Preop Daily Patient Steroid Histological Postop Steroid No. Provisional Diagnosis CRP ESR Dose (mg) Findings Dose (mg) AR, unknown origin AR, unknown origin 3+ AR, unknown origin AR, aortitis, - AR, unknown origin - AR, MR, possible aortitis 3+ AR, possible aortitis AR, aortitis Active, 2+ Inactive Active, 2+ Inactive lo+ 5 1 lo+ 5 1 Scoring in this table: - = negative; 1 + = mild; 2+ = moderate; 3+ = severe. ClU = C-reactive protein; ESR = erythrocyte sedimentation rate (one hour); AR = aortic regurgitation; MR = mitral regurgitation. tients diagnosed to have aortitis syndrome (Table 2). Prednisone was administered in a daily dose of 5 to 7.5 mg depending on the degree of inflammatory reaction. The ESR was measured; the mean level was 16.8 mm in the patients given steroids preoperatively and 44.3 mm in those without steroids before operation. All of the patients seen after Patient 3 were administered steroids postoperatively. Patient 2 underwent aortic valve replacement in August, 198, because of severe orthopnea due to congestive heart failure. He was asymptomatic without steroid therapy except for moderately positive tests for inflammation. Aortic valve replacement was repeated in December, 198, because of dehiscence of the prosthetic valve. At his last operation in April, 1984, there was aneurysmal dilatation of the anterior aortic root involving the annulus attachment of the prosthetic valve. The aneurysm was resected, and the prosthetic valve was reattached with pledgeted mattress sutures of 2- Tevdek from the left ventricular side to the outside of the aortic wall. The patient was discharged with mild exertional dyspnea, and since then has been on a regimen of 7.5 mg of prednisolone daily. Histological Findings A segment of the aortic wall was examined histologically in 8 patients, and active inflammation was seen in 6. Two patients not given steroids before operation (Patients l and 8) showed abundant infiltration of inflammatory cells, mainly lymphocytes and plasma cells, extending from the media to the thickened adventitia. The medial elastic fibers also exhibited marked destruction in those patients. Comment Historically, aortic regurgitation frequently was caused by rheumatic heart disease. Recently, however, fewer patients have had rheumatic fever, and the etiology of aortic regurgitation often is unknown before operation. Idiopathic aortitis is seen more frequently in Japan than in other countries [l, 21. The cardiovascular complications include stenosis of peripheral arteries, renal hypertension or dysfunction, descending aortic aneurysms, and aortic regurgitation [3-51. Pathological findings in aortic regurgitation with aortitis are inflammations involving the ascending aorta, and the aortic annulus dilates because of the weak tone of the aortic wall [6]. Aortic regurgitation develops because the aortic valve cusps become uncoapted [ 71. The patients often are young women [3, 61, and the symptoms are diverse. Since 198 when Takayasu (81 initially described the condition, others [3, 4, 9, 11 have provided additional symptoms: renal dysfunction, hypertension, and chest pain due to either aortic regurgitation or dissecting aortic aneurysm. Although the aortic regurgitation associated with aortitis is seen in 5 to 19% of patients, it rarely necessitates aortic valve replacement [ll]. However, when the ascending aorta is involved and symptoms of angina or congestive heart failure occur because of aortic regurgitation due to uncoapted leaflets, the aortic valve should be replaced. Sometimes the lesions involve the orifice of the coronary artery. Hall and associates [3] reported that angina was seen in 16% of 32 North American patients with Takayasu s aortitis. In our series, 9 patients complained of angina and in 6 of them, there was stenosis of the coronary orifice. Symptoms of congestive heart failure also were seen in 9 patients. In 3 of them, deterioration in these symptoms before operation was associated with laboratory evidence of worsening inflammatory reactions. Measurements of inflammatory reactions, such as the ESR or the CRP level, were always positive during the active phase of the lesion. There was good correlation between clinical evidence

5 185 Isomura et al: Aortitis with Aortic Regurgitation of active inflammation and histological evidence of inflammation of the aortic wall. The inflammatory cells consisted of lymphocytes and plasma cells infiltrating from the adventitia to the media. Few reports have addressed the use and effectiveness of steroid therapy for aortitis [3, 12, 131. In our series, patients were given steroids before operation if they showed typical vascular lesions and an abnormal ESR in addition to aortic regurgitation. Although the disease may involve a genetic factor [14], the diagnosis should be suspected in patients with aortic regurgitation of unknown etiology during an abnormal inflammatory reaction [15]. Steroid therapy after operation carries a risk of critical side effects, such as infection or delayed wound healing. We, however, have observed no such complications in 8 patients treated with prednisone after operation. In contrast, 1 patient who underwent aortic valve replacement during the active phase of the aortitis and received no postoperative steroid therapy, had valve dehiscence. We suggest that postoperative steroid therapy is possibly important for patients with aortitis and may be continued until the inflammatory reaction becomes negative. All of our patients required aortic valve replacement. The prosthetic aortic valve was implanted with pledgeted sutures through the aortic annulus. If the annulus seems fragile, the stitch should be placed through the aortic wall to prevent dehiscence of the prosthetic valve. Even though the aortic wall is always thick, it is very fragile, and attention should be paid to close the incised aortic wall carefully. We usually use 4- Prolene to avoid dissection of the aortic wall. If the wall has already dissected, it should be reconstructed before closure of the aortotomy. Narrowing of the coronary ostium was seen in 5 patients. In such patients, the angina may be due not only to aortic regurgitation but also to stenosis of the coronary artery orifice. As a result, reconstruction of the orifice is necessary in some patients at the time of aortic valve replacement. In conclusion, aortic regurgitation due to aortitis can be difficult to diagnose prior to operation if there are no associated vascular lesions. However, symptoms occurring in young patients with hypertension or renal dysfunction can suggest the diagnosis. Aortic valve replacement is required for aortic regurgitation even though the valve leaflets themselves exhibit only mild lesions. In these patients, the aortic wall is always thick but fragile, and much care should be paid when closing the aortotomy. The coronary ostia sometimes require recon- struction to prevent stenosis of the orifice. Our experience suggests the possible importance of preoperative and postoperative steroid therapy in the management of these patients. We thank Dr. S. J. Galli and Dr. I. M. Rebeyka for reviewing the manuscript. References 1. Ueda H, Ishikawa K, Okabayashi A, et al: Clinicopathology of aortitis syndrome. Saishin Igaku 23:181, Nakao K, Ikeda M, Kimata S, et al: Takayasu s arteritis: clinical report of 84 cases and immunological studies of seven cases. Circulation 35:1141, Hall S, Barr W, Lie T, et al: Takayasu arteritis: a study of 32 North American patients. Medicine 64:89, Sunamori M, Hatano R, Yamada T, et al: Aortitis syndrome due to Takayasu s disease: a guidance for the surgical indication. J Cardiovasc Surg (Torino) 1743, Abe K, Miyazaki S: Clinical aspect of aortitis syndrome with special reference to the relation between prognosis and hypertension. Jpn Circ J 46:19, Gore I: Inflammatory lesions of aortitis: pulseless disease. In Gould SE (ed): Pathology of the Heart and Blood Vessels. Springfield, IL, Thomas, 1968, pp Morooka S, Takeda T, Saito Y, et al: Dilatation of the aortic valve portion in aortitis syndrome: angiographic evaluation of 7 patients. Jpn Heart J 22:517, Takayasu M: Strange change of retinal central vessel: a case report. J Jpn Ophthalmol 12:554, Caccamise WC, Whitman JF: Pulseless disease: preliminary case report. Am Heart J 44:629, Spittel JA Jr, Siekert RG: Anticoagulant therapy of a patient with aortic arch syndrome. Proc Mayo Clin 32:723, DuCailar C, Thurmond A, Semler H, Starr A: Aortic valve replacement for acute Takayasu s disease. Ann Thorac Surg 43:12, Fraga A, Mintz G, Valle L, Flores-Izquierdo G: Takayasu s arteritis: frequency of systemic manifestations (study of 22 patients) and favorable response to maintenance steroid therapy with adrenocorticosteroids (12 patients). Arthritis Rheum 115:617, Ishikawa K: Survival and morbidity after diagnosis of occlusive thromboaortopathy (Takayasu s disease). Am J Cardiol 47126, Tanabe T, Yokota A, Yasuda K Pathogenesis and surgical treatment of aortitis syndrome. Jpn Circ J 46:194, Akikusa B, Kondo Y, Muraki N: Aortic insufficiency caused by Takayasu s arteritis without usual clinical features. Arch Pathol Lab Med 15:65, 1981