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a Department of Cardiac Surgery, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
b Department of Pathology, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Accepted for publication July 9, 2008.
* Address correspondence to Dr Pacini, c/o Unità Operativa di Cardiochirurgia, Università degli studi di Bologna, Policlinico S. Orsola-Malpighi Via Massarenti, 9, Bologna, 40138, Italy (Email: dpacini{at}hotmail.com).
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Abstract |
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Methods: Thirty-nine patients (4.9%) were affected by aortitis (mean age, 72.6 ± 9.6). There were 24 women (61.5%). Thirty-four (87.2%) were operated on because of aneurysms and 5 because of dissection. In all cases the diagnosis of aortitis was incidental and was made on the basis of histopathologic findings.
Results: Histologically, there were 30 cases of giant cell aortitis (76.9%), 3 inflammatory aneurysms (7.7%), 2 cases of aspecific lymphoplasmacellular aortitis (5.1%), 1 of Takayasu aortitis, 1 of systemic erythematosus lupus–associated aortitis, and 1 of Behçet's disease–associated aortitis. The only case of infectious aortitis was a syphilitic aortitis. In 79.5% of cases, inflammatory infiltrates were moderate to severe in degree; the most widespread inflammation was seen in Takayasu aortitis, systemic erythematosus lupus–associated aortitis, and in Behçet's disease. The overall in-hospital mortality was 10.3% (4 of 39 patients). Neurologic complications occurred in 4 patients (10.3%).
Conclusions: During surgery of the thoracic aorta, an inflammatory etiology of aneurysms is found in almost 5% of cases. The inflammatory process is in a histologically advanced phase, often with systemic development. Surgery can be associated with high morbidity and mortality.
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Introduction |
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TopAbstractIntroductionPatients and MethodsResultsCommentReferences |
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The overall incidence of aortitis is not easy to evaluate from the literature: in the United States, the most frequent form, giant cell arteritis with aortic wall involvement in 15% of cases, is reported as 15 to 30 cases per year per 100,000 of the population older than 50 years of age [1, 3]. In the literature, there are few data including histopathologic examinations of large case records of surgical aortic specimens. In these few papers, the incidence of aortitis is extremely variable, ranging from 1% to 22% [4–7], and Homme and colleagues [8] recently identified active noninfectious aortitis in 9% of 513 patients with surgically resected ascending aortic aneurysms.
A separate entity described by Walker and associates in 1972 [9] in the abdominal aorta and later also in the thoracic tract is inflammatory aortic aneurysm, whose incidence varies from 3% to 11% in the cases operated on for abdominal aortic aneurysms [1, 10]. Diagnosis is difficult with only noninvasive imaging studies, so histopathologic examination is required to confirm the presence of the disease.
Surgical treatment leads to an increased risk of tearing as well as of aortic rupture because of the softened tissue. Progressive dilatation of the remaining native aorta may also occur in the absence of radical replacement of the tract involved, or in the case of diffuse development of the disease.
The aims of our study were (1) to detect the incidence of thoracic histologically proven aortitis in a large series of 788 patients affected by thoracic aortic pathology; (2) to describe the surgical and histologic features of inflammatory thoracic aortopathies; and (3) to evaluate the frequency of postsurgical complications and mortality.
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Patients and Methods |
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Thirty-nine patients (4.9%), with a mean age of 72.6 ± 9.6 years and range of 36.6 to 82.1 years, had microscopic evidence of active aortitis. There were 24 women (61.5%; mean age, 73.7 ± 10.7 years) and 15 men (38.5%; mean age, 72.4 ± 8.2 years). Thirty-four (87.2%) were operated on because of aneurysms and 5 (12.8%) because of dissection (3 type A and 2 type B). Informed consent was obtained from all patients, and the study was approved by the institutional review board.
In all cases, the diagnosis of aortitis was made on the basis of histopathologic findings, and was incidental. Two patients were affected by connective tissue disorders (systemic lupus erythematosus [SLE] in 1 and Behçet's disease in the other). In one 48-year-old woman, fever, nights sweats, weight loss, decreased pulses in the extremities, and malaise were present. In another patient, antitreponemal antibodies were positive. In the other 35 patients, no signs or symptoms of systemic inflammatory disease were present at the time of the operation, nor was there a history of giant cell temporal arteritis, Takayasu arteritis, or systemic vasculitis. Before surgery, patients were evaluated by computed tomography (CT), magnetic resonance imaging (MRI), or echocardiography. In all patients, the preoperative CT scan or MRI did not suggest inflammatory aortic disease.
The aneurysmal disease mainly involved the aortic root plus the ascending aorta (21 patients, 53.8%) and the ascending aorta plus the aortic arch (9 patients, 23.1%). Six patients (15.4%) had diffuse involvement of the entire aorta (ascending, arch, descending, and abdominal), and in 2 (5.1%) the aneurysm was limited to the thoracic aorta (ascending, arch, and descending aorta). One patient (2.6%) had an aneurysm only of the aortic arch (Table 1). In all patients we performed total replacement of dilated aortic tracts. The extent of the repair and the associated procedures performed are shown in Table 2.
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The cases of aortitis were classified as noninfective (giant cell arteritis, Takayasu arteritis, aortitis of collagen vascular disease, inflammatory aneurysms, and sarcoidosis) or infective (syphilitic aortitis, tuberculosis, and pyogenic aortitis), as reported by Virmani and Burke [1]. For the cases with apparent lymphoplasmacytic aortitis, additional sections were prepared from three deeper levels in the paraffin blocks to evaluate the presence of giant cells more extensively. Takayasu arteritis and giant cell aortitis were distinguished according to patient age and the presence of two or more established clinical criteria [11–13].
According to a semiquantitative evaluation of the percentage of the entire length of the microscopic aortic wall involved, the extent of inflammatory infiltrates was considered as slight (one third of the surface), moderate (two thirds of the surface), or severe (the whole aortic wall). In addition, on the basis of the predominance of active or healing lesions in the aortic wall, the inflammatory process was defined as active phase (prevalence of widespread inflammatory infiltrates, necrosis, and edema) or healing or healed phase (prevalence of medial fibrous scarring, fragmentation and disruption of medial elastic fibers, fibrointimal thickening, and adventitial scarring).
Follow-Up
Clinical follow-up was available for all 35 discharged patients and ranged from 26 to 125 months (mean, 52 ± 28 months). Information was obtained by examination of the patients or by communication (telephone or mailed questionnaire) with the patients or their referring physicians as well as from the CT, MRI, and echocardiographic evaluations carried out. All patients were followed either by CT scan, MRI, or echocardiography.
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Results |
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The inflammatory infiltrates consisted in all cases of lymphocytes, monocytes, and plasma cells, but they differed in degree and localization in the aortic wall according to type. They were multifocal and predominantly in the medial layer in giant cell aortitis (Fig 1A) and widespread, and localized at the intimomedial junction, in the whole medial layer, and in the adventitia in Takayasu aortitis (Fig 1B) and SLE-associated aortitis.
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Giant cells were always present in giant cell aortitis (Fig 1D), were particularly numerous in Takayasu aortitis (Fig 1E), in which medial and adventitial granulomata were also present (Fig 1F), and were scattered in Behçet's disease.
All cases of inflammatory aortic aneurysms showed complex atherosclerotic plaque, severe parietal inflammatory infiltrates, attenuated media with extensive fibrous scarring and elastic fiber disruption, and severe fibrous thickening of periadventitial tissue. The inflammatory process was active (Fig 2A, 2B) in 19 patients (48.7%) and healing or healed (Fig 2C, 2D) in 20 patients (51.3%).
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Surgical Analysis
Early results
Four patients (10.3%) died after the operation. The causes of hospital death were a stroke in 2 patients (5.1%), bleeding in 1 patient (2.6%), and myocardial failure in 1 patient (2.6%). Three of these 4 had GCA and 1 had SLE. Two patients with GCA underwent aortic root replacement with composite valve graft, and both underwent an associated procedure: 1 had a mitral valve replacement and died of bleeding, the other underwent coronary artery bypass grafting and hemiarch replacement and died of cardiac failure. The third patient underwent ascending aorta and hemiarch replacement associated with coronary artery bypass grafting, and died of a stroke. The patient with SLE was a 55-year-old woman, who underwent composite valve replacement and hemiarch replacement and died on the 10th postoperative day of a stroke.
Neurologic complications occurred in 4 patients (10.3%): 2 patients had a fatal stroke and 2 had transitory dysfunctions that were completely resolved before hospital discharge. Three patients (7.7%) required rethoracotomy, 2 for bleeding and 1 for sternal osteomyelitis. Cardiac complications occurred in 3 patients (2 myocardial failures, 1 myocardial ischemia), and postoperative renal insufficiency requiring dialysis in 2 (5.1%). The patient with syphilitic aneurysm of the aortic arch had severe ventilatory insufficiency owing to tracheomalacia that required stenting of the trachea and the main bronchi [14].
Late results
The follow-up of the 35 discharged patients was 100% complete. No patient underwent corticosteroid or immunosuppressive therapy after diagnosis of inflammatory aortic disease. The actuarial survival rate was 80.7% ± 6.7% and 47.1% ± 20.6% at 5 and 10 years, respectively (Fig 3). There were 5 late deaths (14.3%) during follow-up. Cause of death was unknown in 1 patient, stroke in 1, renal carcinoma in 1, myocardial failure in 1, and rupture of an unidentified abdominal aorta aneurysm in 1. One patient with GCA died of unknown cause 2 years after the operation. One patient, who underwent surgery for ascending aorta and total arch replacement associated with coronary artery bypass grafting, died of a stroke 3 months later: he had an inflammatory aneurysm. One other patient with an inflammatory aneurysm died of renal carcinoma with pulmonary metastases. One patient with GCA died 5 years later of hemorrhagic shock, owing to rupture of an unrecognized abdominal aorta aneurysm. The fifth late death was a 24-year-old man affected by Behçet's disease who underwent a Bentall procedure: he underwent reoperation after 3 years for detachment of the composite valve graft from the aortic annulus, and then underwent a second reoperation for pseudoaneurysm owing to complete detachment of the coronary arteries from the graft. The patient died of cardiac failure 7 years after the initial operation.
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Comment |
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In our study, women were affected slightly more than men (61.5% versus 38.5%); in most of the data reported in the literature women were more frequently or predominantly affected (2:1 to 4:1), although a higher incidence in men [5] has also been reported.
The mean age of our population was relatively high (72.6 ± 9.6 years), as reported by other authors [6, 15, 16]; the youngest patients were affected by collagen syndromes such as SLE and Behçet's disease as well as by Takayasu syndrome.
Our histopathologic results showed that noninfective aortitis is now much more frequent than infective forms (97.4% versus 2.6%). Among the noninfectious forms, our samples confirm that GCA (66.6% of these cases) is the most frequent histopathologic diagnosis (76.9%), as found by others [4, 6, 10, 15], especially among female patients (66.5%) [1].
Moreover, among the 38 patients with noninfective aortitis, the majority (36 patients, 92.3%) had isolated aortitis: in these patients, neither evidence of systemic disease nor known history of temporal arteritis was present at the time of surgery. In the literature, the data regarding the percentage of isolated aortitis vary widely, from 25% to 94.7% [5, 15–18]: as in our population, the high percentage of this type of disease is partly attributable to a less than exhaustive investigation of the presence of systemic inflammatory disease. It is clear that in a large group of patients aortitis is a localized disease.
In the majority of our patients, the morphologic alterations were in an advanced phase at the time of surgery, with severe and widespread lesions in 74.4% of cases, and even more severe in female patients, who also showed widespread destruction of the aortic wall. Inflammatory infiltrates were present in all specimens, and they were extensive and moderate to severe in 80% of cases, particularly in aortitis associated with collagenopathies and with inflammatory aneurysms, in which all cases showed severe infiltrates. However, in 20 (51.3%) patients inflammation was associated with healing aspects.
These pathologic features are the cause of weakening of the aortic wall and of aneurysm formation and evolution. The near-complete disruption of the medial elastic component may induce rupture and dissection, even in small aneurysms. Evans and associates [19] reported a series of 9 patients, 4 (44%) of whom died suddenly of aortic dissection. Other authors cited a high proportion of specimen diagnoses made from ruptured aortic autopsy tissue rather than from surgical specimens [17, 20, 21]. In our patients, aortic dissections were less frequent than in the literature: 5 (12.8%) of 39 patients.
The extension of both the aortic aneurysm and the aortic replacement was limited to the ascending aorta and the aortic arch in the majority of patients (76.9%). Patients with GCA had a higher rate of involvement of the proximal part of the thoracic aorta (90%). In these cases, the Bentall operation is the treatment of choice even though the aortic valve cusps are usually macroscopically normal and histologic examination shows no evidence of inflammatory process [22, 23]. The aortic valve could therefore be safely preserved in patients with functionally normal valve leaflets. We mainly performed the Bentall procedure (18 patients), but in 3 cases we did a valve-sparing operation according to the David technique and none of the latter group required reoperation. In aortitis, the more hemostatic reimplantation procedure should be preferred to remodeling to reduce the risk of bleeding from the proximal suture line. In fact, in the case of inflammatory involvement, the aortic tissue can be fragile, leading to tearing of the suture line. One patient died of uncontrollable bleeding owing to a very fragile aorta even though the suture lines were reinforced with a Teflon felt strip.
In our experience, surgical results of aortic repair seem to be worse in inflammatory aneurysms than in aneurysms caused by other pathologic processes. In the present series, the mortality rate was 23.1%, although in our overall experience it was approximately 10% [24–26]. However, these are not risk-adjusted populations and definitive conclusions cannot be made.
The incidence of neurologic complications (10.3%) was very high in this subgroup of patients who underwent aortic surgery. Two patients experienced a stroke and 2 others had temporary neurologic dysfunction. So far we have performed more than 250 repairs of the thoracic aorta for various diseases using antegrade selective cerebral perfusion, and we have had only 4 cases of stroke: 2 of these were affected by aortitis [24]. We cannot explain this high rate of neurologic complications, but a possible explanation may be that it is related to specific inflammatory disease.
Surgical repair is important in aortitis and lifesaving in the short term, but prognosis and treatment is determined by the underlying cause of disease and pathologic examination may aid in clarifying the diagnosis [27]. Because of the worse results obtained and also because of the aortic wall weakness we found in these patients, we could speculate that, in the case of inflammatory aneurysms, the elective treatment could be performed at a lesser diameter than in noninflammatory ones. Unfortunately, the preoperative diagnosis is very hard: the radiologic imaging (CT and MRI) did not show any suggestion of inflammatory disease. Owing to the fact that preoperative diagnosis is difficult and the incidence of aortitis is by no means negligible, it would be useful to routinely perform a preoperative measurement of C-reactive protein and erythrosedimentation rate in all patients undergoing aortic surgery.
During the follow-up, according to the CT and MRI examinations, there was a minimal growth of the remaining aorta. The freedom from aortic reoperation or recurrent aneurysmal disease was 93.4% ± 4.5% at 5 years and 81.7% ± 11.6% at 10 years. Two patients exhibited a new aneurysm, both at the abdominal tract of the aorta; 1 was operated on 2 years after the first operation, the other died of abdominal aorta rupture 5 years after the first operation. Both patients were affected by GCA. The patient with Behçet's disease underwent two reoperations: one for prosthetic valve detachment and one for detachment of the coronary arteries. In patients with aortitis syndrome, paravalvular leakage or valve detachment and anastomotic false aneurysm are the most serious and frequent complications [28]. The major factor responsible for the high incidence of these complications is persistent inflammation. For this reason, adequate monitoring of inflammation is extremely important. However, the inflammation is sometimes difficult to identify before surgery, or in other cases surgery is necessary during the active phase. Special surgical techniques are required in such patients to prevent paravalvular leakage or prosthetic graft detachment.
Histopathologic examination of surgical aortic specimens is very important, because it not only can identify the presence of inflammatory disease but also provides evidence of aortic inflammation activity and defines the infectious or noninfectious etiology, which is important to adopt an appropriate antimicrobial or immunosuppressive therapy [16, 27].
Medical treatment of aortitis consists mainly of corticosteroids, but there is no consensus on the dose or duration of steroid treatment after a histologic diagnosis of arteritis. Most physicians recommend steroid treatment in the active phase of the disease. Prolonged steroid treatment has, however, been known to affect the connective tissues by inhibiting chondroitin sulfate and formation of granulation tissue, and would probably influence the disintegration of connective tissue of the media aggravated by steroid-induced long-standing hypertension [29]. Further studies are required to define the utility of medical treatment in aortitis, particularly the optimal immunosuppressive regimen. No patient in our population was treated with either steroid or immunosuppressive therapy.
During surgery of the thoracic aorta, an inflammatory etiology of aneurysms is found in almost 5% of cases. The inflammatory process is in a histologically advanced phase with active lesions, often with systemic involvement. Surgery can be associated with high morbidity and mortality. Pharmacologic therapy, which is so often forgotten because of the absence of a histopathologic diagnosis, can be based on steroid and immunosuppressive drugs. In addition, the high incidence of diffuse aortic involvement and the possibility of further, long-lasting development of new aneurysms in different aortic tracts call for close monitoring of patients by CT and MRI. Histologic examination is an important diagnostic tool in aortic surgery, as it is relevant to both short-term and long-term results.
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