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Ann Thorac Surg 2005;79:1512-1517
© 2005 The Society of Thoracic Surgeons

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Original articles: Cardiovascular

Surgical Treatment of Ascending Aortic Aneurysms in Patients With Giant Cell Aortitis

Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota

Accepted for publication October 12, 2004.

^_* Address reprint requests to Dr Zehr, Division of Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (E-mail: zehr.kenton{at}mayo.edu).

	Abstract

Top Abstract Introduction Patients and Methods Results Comment References

 
BACKGROUND: Giant cell aortitis is a rare cause of ascending aortic aneurysm disease despite giant cell arteritis being a common cause of vasculitis. We evaluated an 8-year experience with surgical repair with regard to preoperative variables, extent of disease, required surgical procedures, and the propensity to develop additional great vessel aneurysms.

METHODS: Thirty-seven patients (29 female, 8 male; aged 69.6 ± 9.5 years) were operated on from 1995 to 2002. Ten (27%) patients had a history of steroid treatment for temporal arteritis or polymyalgia rheumatica 8.9 ± 3.9 years before. Nineteen (51%) patients had +3 or +4 aortic regurgitation. Maximal aneurysm size was 6.1 ± 0.8 cm. Thirty (81%) patients underwent polyethylene terephthalate fiber (Dacron) tube graft replacement of the ascending aorta, 4 (11%) had a modified Bentall procedure, 2 (5%) had a valve-sparing aortic root reconstruction, and 1 (3%) had aortorrhaphy. Twenty-two (59%) patients required 22 ± 9 minutes of hypothermic circulatory arrest for hemiarch or complete aortic arch replacement. Twenty-six (70%) patients had concomitant cardiac procedures.

RESULTS: There was no early mortality. Morbidity was reexploration for bleeding in 3 (8%) patients, stroke in 3 (8%), left vocal cord paralysis in 2 (5%), renal failure in 2 (5%), and gastrointestinal bleeding in 1 (3%). Mean follow-up was 2.8 ± 2.3 years. Four-year actuarial survival was 74% (95% confidence interval, 57% to 94%). Other descending or abdominal aortic or great vessel aneurysms occurred in 17 (46%) patients. Four patients had prior aneurysm surgery, 8 are monitored with aneurysms, and 5 underwent repair of an aneurysm in the follow-up period. Of 8 late deaths, 3 were caused by complications of a descending thoracic aneurysm. No patient required replacement of a native aortic valve that was preserved during the initial operative procedure.

CONCLUSIONS: Ascending aortic aneurysms caused by giant cell aortitis can involve the aorta from the aortic root through the aortic arch, thus requiring a tailored operative approach. The aortic valve tissue is spared from the pathologic process. Other aneurysms of the aorta and great vessels occur in nearly half of patients. Frequent surveillance of the remaining aorta is mandatory.

	Introduction

Top Abstract Introduction Patients and Methods Results Comment References

 
Giant cell arteritis is a granulomatous inflammatory disease of medium and large sized arteries. It was first described clinically in 1890 by Hutchinson [1] and histologically in 1932 by Horton and associates [2] after biopsy of a patient presenting with a swollen, painful temporal artery. The general population prevalence has been reported to be 1% in a large autopsy series [3]. Machado and colleagues [4] have reported the average annual incidence of temporal arteritis to be 17/100,000 person-years in our local patient population in Olmsted County, Minnesota. Of the 96 patients with giant cell arteritis, 78 (81%) were female. The median age was 75 years (range, 56 to 92 years).

The temporal arteries are the most commonly affected. However, extracranial manifestations of giant cell arteritis have been reported with varying frequency in the aorta and more rarely in visceral vessels. Evans and coworkers [5] observed the 96 patients in our local county with temporal arteritis. Nine of the 94 (9.6%) patients with no aneurysmal disease (2 had a concurrent aneurysm at the initial diagnosis) had a thoracic aneurysm in a median of 5.8 years. The ascending aorta and aortic arch are the most commonly affected extracranial portions of the vascular tree. Acute presentation is common, including aneurysm rupture, dissection accompanied with severe aortic regurgitation, stroke, and myocardial infarction [6–9]. Standard therapy has been steroid therapy, but there is no consensus concerning duration and dose. It has been theorized that aortic aneurysm enlargement can be arrested by chronic steroid therapy. Clearly patients develop aortic aneurysms despite ongoing treatment for giant cell arteritis [5]. Active disease is usually treated long-term with tapering doses for months to years. It is not clear how efficacious steroid treatment is in preventing further vascular complications.

The objective of this study was to examine surgical results in the current era and to assess the stability of the remaining aorta during follow-up.

	Patients and Methods

Top Abstract Introduction Patients and Methods Results Comment References

 
Patients
Thirty-seven patients with an aneurysm involving the ascending aorta caused by giant cell aortitis underwent surgery at the Mayo Clinic, Rochester, MN, between January 1995 and December 2002. A retrospective review of our surgical database and patient medical records was performed. Follow-up information was obtained by questionnaire or telephone calls to assess current status of health, medications, and any significant medical events. The primary indication for surgery in all 37 patients was a maximum aortic diameter greater than 5.5 cm or symptomatic aortic regurgitation.

Surgical Procedures
All patients underwent a standard median sternotomy. Cardiopulmonary bypass was initiated after arterial cannulation in the ascending aortic in 31 (83.8%) patients and the femoral artery in 6 (16.2%) patients.

Patients with isolated ascending aortic dilatation above the sino-tubular junction underwent replacement of the ascending aorta with a polyethylene terephthalate fiber (Dacron) tube. A modified Bentall procedure using a composite valve conduit was used to replace the aortic root in patients with sino-tubular junction dilation and an abnormal aortic valve. Patients with preserved sino-tubular junction dimensions with an abnormal valve had an aortic valve repair or replacement in addition to replacement of the ascending aorta. If the valve was deemed normal with dilatation of the root, a valve-sparing root reconstruction was performed with either resuspension of the valve or reconstruction of the sinuses and reimplantation of the coronary arteries. Patients with involvement of the arch required deep hypothermic circulatory arrest for partial or complete arch replacement. All 4 patients undergoing complete arch replacement had an elephant trunk procedure. Associated procedures were performed in 26 (70.3%) patients with cardioplegic arrest.

Statistical Analysis
Postoperative survival was estimated by the Kaplan-Meier method. Overall survival was compared with the expected survival of persons of the same age and sex, as derived from vital statistics for the West North Central region of the United States. The statistical significance of observed versus expected survival was assessed with a one-sample log-rank test. The associations of potential risk factors to survival were assessed with log-rank tests. Cox proportional hazard models were used to evaluate continuous variables. Data were expressed as mean ± standard deviation, and statistical significance was considered at p less than 0.05.

	Results

Top Abstract Introduction Patients and Methods Results Comment References

 
Demographics, presenting symptoms, and significant comorbidities are listed in Table 1. Seventeen (45.9%) patients were in New York Heart Association class III or IV. Nineteen (51%) had greater than grade II aortic regurgitation. Moderate mitral and tricuspid regurgitation was documented in 3 patients each. Two patients had significant aortic stenosis. Three patients had a prior thoracoabdominal aneurysm repair, of which 2 patients underwent emergent surgery because of a leaking aneurysm. One patient had previous coronary artery bypass grafting, and another had a femorofemoral artery bypass. Only 8% of patients had biopsy-proven giant cell arteritis before presentation. Evidence of a past history of temporal arteritis or polymyalgia rheumatica syndrome was found in 10 (27%) patients. The mean duration between diagnosis to aortic surgery was 8.9 ± 3.9 years. All of these patients had received steroid therapy.

View larger version (105K): [in this window] [in a new window]   Fig 1. A 7-cm ascending aortic aneurysm involved with giant cell aortitis. Note the glistening cobblestone appearance.

View larger version (147K): [in this window] [in a new window]   Fig 2. Inside view of the coronary buttons and residual involved aorta in a patient with aortic root involvement who was prepared for a valve-preserving aortic root reconstruction. The inflammatory process is grossly evident in the residual aorta, but the morphology of the valve cusps is preserved.

View larger version (94K): [in this window] [in a new window]   Fig 3. Computerized tomographic image showing an ascending aortic aneurysm involved with giant cell aortitis. Note the left posterior thickening of the aorta from the inflammatory process (arrow).

View larger version (120K): [in this window] [in a new window]   Fig 4. Photomicrograph of cross-section through aortic wall showing near-complete disruption of the elastic fibers and media by the inflammatory giant cell process.

View larger version (11K): [in this window] [in a new window]   Fig 5. Survival curve of patients undergoing surgical repair compared with the expected survival of our age- and sex-matched regional population.

Postoperative Steroid Treatment
Thirteen patients received steroids postoperatively with a 6-month to 3-year taper depending on the inflammatory markers and presence or absence of symptoms. Of the patients who received steroids, 5 are being monitored with a thoracic aneurysm.

	Comment

Top Abstract Introduction Patients and Methods Results Comment References

 
Giant cell arteritis can effect the entire vascular tree. Lie [10] has documented that in 72 patients with extracranial disease, the ascending aorta and aortic arch were involved in 39% of cases, the subclavian and axillary arteries in 28%, and the femoropopliteal arteries in 18%. Twenty-five percent had associated temporal arteritis. This ratio was similar in our series. Whereas only 8% of patients had biopsy-proven temporal arteritis in the past, a total of 10 (27%) patients had clinical evidence of temporal arteritis or polymyalgia rheumatica.

The vast majority of patients with temporal arteritis present with symptoms of headache, diplopia, vision loss, facial pain, and jaw claudication. These symptoms can be pathologically correlated to the occlusive disease caused by the inflammatory process [11]. In patients with axillary or femoropopliteal disease, similar occlusive presenting symptoms are common [10, 12]. The presentation of giant cell aortitis is in sharp contrast to patients with temporal arteritis. Only 6 (16%) patients in our series presented with symptoms related to their ascending aortic aneurysms. A large proportion of patients were asymptomatic at presentation with the aneurysm discovered incidentally on routine physical examination or during work-up of an unrelated pathologic process. Of 9 patients who presented with breathlessness on exertion, in 7 the symptoms could be attributed to significant aortic insufficiency or associated coronary artery disease. As is common, 14 of the 19 with more than moderate aortic insufficiency were asymptomatic.

The question of whether to serially monitor all patients with temporal arteritis or polymyalgia rheumatica for the development of a thoracic aneurysm must be raised. In our study, evidence of a past history of temporal arteritis or polymyalgia rheumatica was found in 10 of the 37 (27%) patients. All were treated with steroids for variable periods (6 months to 3 years) depending on the response based on markers of inflammation. The mean duration of diagnosis of temporal arteritis to aortic surgery was 8.9 ± 3.9 years. In our series, most patients had silent vasculitis. In symptomatic patients, the symptoms were related to the aneurysm or the associated aortic regurgitation. This was true even for patients who were previously treated for symptomatic temporal arteritis. This suggests that patients with a history of temporal arteritis should be serially assessed for the development of large vessel disease. Evans and colleagues [5] have done this in our local population. They found 11 of 96 (11.5%) patients had (2 patients) or developed a thoracic aneurysm (9 patients) during a median time of 5.8 years after the diagnosis of temporal arteritis. Of interest, it has been shown by Lie [10] that all 72 patients with extracranial giant cell arteritis described had evidence of temporal arteritis, 67 by biopsy before, concurrent with, or after the diagnosis of aortic and extracranial large vessel giant cell arteritis, 5 by clinical criteria. Lie's study provides evidence that cranial symptoms are often absent in patients with aortitis despite biopsy-proven giant cell involvement of the temporal arteries. Therefore, the presence of symptoms is a suboptimal endpoint to initiate a screening process for aortitis. We recommend that all patients with temporal arteritis are screened for large vessel disease.

Patients with aneurysms caused by giant cell aortitis carry a high incidence of catastrophic complications. Forty-four percent of the patients (4 of 9) from the Olmstead County population study died suddenly of aortic dissection [5]. Liu and associates [9] reported on a series of 23 patients with giant cell aortitis presenting with dissection; 46% of patients presented catastrophically. Of these, the 2-week mortality was 80%. Others have shown a high proportion of specimen diagnoses made from ruptured aortic autopsy tissue as compared with surgical specimens [8, 10, 13, 14]. Our series corroborates these data. Seventeen patients in the present series had aneurysmal involvement of the descending or thoracoabdominal aorta. Five of these patients have died. Three were caused by complications of a thoracic aortic aneurysm, 2 had documented rupture, and 1 had a localized rupture with a thoracic hematoma that contributed to the development of disseminated intravascular coagulation. All 3 patients had aneurysms of less than 6 cm in diameter. One patient died suddenly, and an autopsy was not performed. The patient numbers are too small to conclude that these aortas are more unstable than in patients with aneurysmal disease caused by atherosclerotic disease. The histology of this disease suggests that patients often have a near-complete disruption of the elastic medial layer [9–11, 13–15] (Fig 4). This may predispose patients to rupture and dissection before the aneurysm achieves the standard sizes for usual intervention. Eklund and coworkers [16] reported on a patient who presented with an aortic rupture 3 cm above the valve annulus with a normal sized aorta but marked thinning of the aortic media. The patient had been treated for 5 years with a maintenance dose of 5 mg/d of prednisolone after achieving a normal erythrocyte sedimentation rate. There is no doubt that close surveillance of the remaining aorta in this patient population is necessary. We recommend computed tomographic scanning on a yearly basis with surgical consideration given at smaller dimensions similar to patients with connective tissue disorders.

Our surgical results were similar to surgery for aneurysms caused by other etiologies. The same algorithm concerning surgery was applied. The usual indication was an ascending aneurysm greater than 5.5 cm or severe aortic regurgitation. The aortic arch or ascending aneurysm was usually cannulated, and femoral cannulation was occasionally performed in patients with tenuous aneurysmal walls. In retrospect, we do not believe there is any contraindication to axillary cannulation, and this procedure should be considered in patients in whom arch reconstruction is required. The diagnosis was nearly always made by histologic examination. As we gained experience with the clinical entity, a high index of suspicion could be obtained by the gross appearance of the aorta at the time of surgery (Fig 1). If the aneurysm extended into the aortic arch, then replacement was extended to include a partial or complete arch reconstruction. All complete arch reconstructions were and should be done as an elephant trunk procedure in anticipation of the possible need for future descending aortic replacement. The aortic root was involved in only 6 (16%) patients. As the patient population was older, valve-sparing aortic root reconstruction was performed in only 2 patients; neither has required reoperation. In the 4 patients who had their native valves excised, the valve cusps showed no histologic evidence of an inflammatory process. Therefore, the valve can safely be preserved in patients with functionally normal valves. One patient underwent aortorrhaphy. This was performed before finding out the diagnosis. We do not recommend this procedure if there is an index of suspicion for giant cell aortitis.

There is no consensus on the dose and duration of steroid treatment after a histologic diagnosis of giant cell arteritis. Most clinicians recommend treatment with steroids in the active phase of disease. The active phase is determined by either symptomatic vasculitis or elevated markers of inflammation. Our practice is to start prednisolone in a dose of 40 to 60 mg/d in adults and continue for 6 months to 2 years depending on the response. Cyclophosphamide, azathioprine, or dapsone may be used with steroids if the erythrocyte sedimentation rate does not respond. In our series, 13 patients received steroid therapy as per the guidelines mentioned. Of these 13 patients, 5 had developed or had progression of an aneurysm in the remaining aorta despite steroid therapy. Of the 8 patients who died during follow-up, only 1 had received steroids after surgery. All 3 patients who had fatal complication of a thoracic aortic aneurysm during follow-up had not received steroids. Whether aneurysmal dilatation of the remaining aorta and great vessels can be prevented by an aggressive regimen of steroids or antineoplastic agents is unclear. Nevertheless, we have continued an aggressive treatment approach in patients with evidence of active disease.

Giant cell aortitis most commonly presents as an ascending aortic aneurysm involving the ascending aorta at and above the sino-tubular junction and frequently extends into the aortic arch. There is often associated central aortic insufficiency related to effacement of the sino-tubular junction. The aortic valve tissue appears to be spared from the vasculitic process. A past history of temporal arteritis or polymyalgia rheumatica is inconsistent. Assessment of extracranial disease should be the standard of care for patients with these syndromes. A surgical strategy of tailoring the operation to address the aortic disease and aortic valve insufficiency can be done with low morbidity and mortality. Treatment with steroids based on assessment of the acute inflammatory process does not seem to protect from aortic involvement. The high incidence of involvement of other parts of the aorta portends close surveillance of the remaining aorta.

	References

Top Abstract Introduction Patients and Methods Results Comment References

 

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