Post-operative outcomes of inflammatory thoracic aortitis: a study of 41 patients from a cohort of 1119 surgical cases.

Aortitis is found in 2-12% of thoracic aortic aneurysm repair/replacement surgeries. Yet little is known about such patients' post-operative outcomes or the role of post-operative corticosteroids. The study was undertaken across three tertiary referral hospitals in Sydney, Australia. Prospectively collected data for all thoracic aortic repair/replacement patients between 2004 and 2018 was accessed from a national surgical registry and analysed. Histopathology records identified cases of inflammatory aortitis which were subclassified as clinically isolated aortitis (CIA), giant cell arteritis (GCA), Takayasu (TAK) or other aortitis. Between-group outcomes were compared utilising logistic and median regression analyses. Between 2004 and 2018, a total of 1119 thoracic aortic surgeries were performed of which 41 (3.7%) were inflammatory aortitis cases (66% CIA, 27% GCA, 5% TAK, 2% other). Eight out of 41 (20%) aortitis patients received post-operative corticosteroids. Compared to non-aortitis patients, the aortitis group was predominantly female (53.7% vs. 28.1%, p < 0.01), was older (mean 70 vs. 62 years, p < 0.01) and had higher prevalence of hypertension (82.9% vs. 67.1%, p = 0.03) and pre-operative immunosuppression (9.8% vs. 1.4%, p < 0.01). There was no difference (p > 0.05) between aortitis and non-aortitis groups for 30-day mortality (7.3% vs 6.5%), significant morbidity (14.6% vs. 22.4%), or infection (9.8% vs. 6.4%). Outcomes were similar for the non-corticosteroid-treated aortitis subgroup. Histologic evidence of inflammatory thoracic aortitis following surgery did not affect post-operative mortality or morbidity. Withholding corticosteroids did not adversely affect patient outcomes. These findings will assist rheumatologists and surgeons in the post-operative management of aortitis.

Etiology, Diagnosis and Management of Aortitis.

Aortitis includes conditions with infectious or non-infectious etiology, characterized by inflammatory changes in one or more layers in aortic wall. Age at onset, geographic predilections, distribution and pattern of involvement in aorta, its branches and pulmonary arteries, and systemic associations provide a clue to etiology. Clinical presentations are often non-specific. An integrated approach including clinical, laboratory and imaging assessment is essential to confirm diagnosis and plan treatment. Assessment of disease activity is the key as it influences timing and outcome of treatment. Markers of activity include clinical, laboratory and imaging. Medical management remains the first-line therapy. Revascularization is indicated in the presence of hemodynamically significant stenosis and inactive disease. In the presence of flash pulmonary edema, left ventricular dysfunction or hypertensive encephalopathy, revascularization is performed irrespective of disease activity. Endovascular management is favored over surgery due to its high success and low restenosis rates. Symptomatic aneurysmal disease is usually managed by surgery.

Giant cell arteritis in a patient with aortic dissection: a case report.

Aortic lesions, such as an aortic aneurysm, are known as a late complication that usually occurs several years after the onset of giant cell arteritis. Here, we report a rare case of large-vessel giant cell arteritis in a patient with aortic dissection. A 71-year-old man presented with acute back pain and was diagnosed with aortic dissection, Stanford type A, and he underwent elective ascending aortic replacement. Further studies showed that the resected ascending aorta had aortic dissection and multinucleated giant cell granulomas; the granulomas were located in the media near the intima with partial destruction of the internal elastic lamina; there was no stenosis of the feeding blood vessel or fibrosis of the adventitia as observed in Takayasu arteritis; other types of vasculitis were considered unlikely based on the symptoms and laboratory data. The patient was further diagnosed with giant cell arteritis, which was classified as a large vessel vasculitis along with Takayasu arteritis at the Chapel Hill Consensus Conference in 2012. This is a rare case of giant cell arteritis diagnosed in a patient with aortic dissection. The differences in histopathological findings between Takayasu arteritis and giant cell arteritis are discussed.

Clinical Experience of Aortic Valve Surgery in Patients With Aortitis Disease.

OBJECTIVE: To investigate single center's clinical experiences of aortic valve surgery for aortitis patients. METHODS: From January 2010 to December 2018, 15 patients with aortitis disease were treated in our center. Among them, there were 7 males and 8 females with an average age of 48.5 ± 13.9 (29-76) years. Six patients were diagnosed as giant cell arteritis, 2 as Takayasu arteritis (Arteritis Group, N = 8) and 7 as Behcet's disease (BD Group, N = 7). Aortic valve surgery includes valvuloplasty, valve replacement, and root replacement (Bentall procedure). RESULTS: There were 15 cases with 19 operations, 8 cases in the Arteritis Group received 8 operations while 7 cases in the BD Group received 11 operations, including 4 redo operations. Preoperative patients' aortic valve regurgitation degree, diameter of ascending aorta and left ventricular ejection fraction were similar between the two groups. The type of aortic valve surgery also was no different. Cardiopulmonary bypass time of the BD Group seemed longer than the Arteritis Group but no different, the same as clamp time. Mechanical ventilation time is longer in the BD Group. The morbidity and mortality were similar, but the BD Group had significantly higher incidence of redo operations because of postoperative paravalvular leak or valve insufficiency (Arteritis Group versus BD Group, 0% versus 57.1%, P = .026). CONCLUSIONS: Clinical diagnosis and management of aortic valve patients with arteritis require comprehensive considerations. For aortitis patients with aortic valve surgery, special surgical techniques can be used to reduce the risk of prosthetic valve detachment.

Annuloaortic Ectasia and Arteritis: Clinical Features, Treatments of Choice, and Causative Relations.

The roles that aortitis plays in the development of annuloaortic ectasia (AAE) remain uncertain, while clinical features of AAE in arteritis are largely unknown. This study was designed to highlight the clinical features of AAE, the treatments of choice, and the causative relations between aortitis and AAE. The morphology of the aortic valve leaflets was normal in half of the patients, while the valves were thin and overstretched in the other half. Most patients had an aortic aneurysm. Half of the patients had severe aortic valve insufficiency, and one-quarter of them had dilation of the sinuses of Valsalva. Takayasu arteritis was prone to develop coronary artery lesions, whereas giant cell arteritis were not. Aortic branch lesions in Takayasu arteritis were stenotic or occlusive in 92.9% of the patients, while in giant cell arteritis, they were all dilated lesions. Most patients (94.7%) required surgical treatment with steroid therapy. However, long-term follow-up results showed a higher anastomotic dehiscence rate, particularly in patients with Takayasu arteritis. Further morphometric and pathological research on AAE in arteritis should be undertaken, and more feasible measures should be warranted for preventing postoperative anastomotic dehiscence.

Annuloaortic ectasia and arteritis: clinical features, treatments of choice, and causative relations

Abstract The roles that aortitis plays in the development of annuloaortic ectasia (AAE) remain uncertain, while clinical features of AAE in arteritis are largely unknown. This study was designed to highlight the clinical features of AAE, the treatments of choice, and the causative relations between aortitis and AAE. The morphology of the aortic valve leaflets was normal in half of the patients, while the valves were thin and overstretched in the other half. Most patients had an aortic aneurysm. Half of the patients had severe aortic valve insufficiency, and one-quarter of them had dilation of the sinuses of Valsalva. Takayasu arteritis was prone to develop coronary artery lesions, whereas giant cell arteritis were not. Aortic branch lesions in Takayasu arteritis were stenotic or occlusive in 92.9% of the patients, while in giant cell arteritis, they were all dilated lesions. Most patients (94.7%) required surgical treatment with steroid therapy. However, long-term follow-up results showed a higher anastomotic dehiscence rate, particularly in patients with Takayasu arteritis. Further morphometric and pathological research on AAE in arteritis should be undertaken, and more feasible measures should be warranted for preventing postoperative anastomotic dehiscence.

Outcomes Among 196 Patients With Noninfectious Proximal Aortitis.

OBJECTIVE: Noninfectious aortitis may occur in the context of a recognized systemic disease or as a topographically limited lesion without systemic features, which is called clinically isolated aortitis (CIA). This study was undertaken to better define and stress the limitations of this diagnostic category in a large population of patients in a single center dedicated to aortic diseases and to suggest recommendations for care. METHODS: Records of patients undergoing thoracic aortic surgery (1996-2012) at the Cleveland Clinic were reviewed to identify 196 patients with histopathologically proven aortitis. Clinical diagnoses (giant cell arteritis [GCA], Takayasu arteritis [TAK], CIA, or Other) were determined at the time of surgery. Clinical features, laboratory findings, and imaging results were recorded throughout the follow-up period. At least 6 months of follow-up data were available for 73 CIA patients. RESULTS: The mean age of the patients at time of surgery was 65.6 years (range 15-88 years); 67% of patients were female, and 90.3% were white. At the time of surgery, 129 patients (65.8%) met criteria for CIA, 42 (21.4%) for GCA, 14 (7.1%) for TAK, and 11 (5.6%) met criteria for other systemic inflammatory diseases. During a mean follow-up period of 56.2 months, 19% of CIA patients developed new symptoms, 45% developed new radiographic vascular lesions, 40% underwent additional vascular surgery, and 12% died (n = 9). Eleven of 73 patients (15%) initially classified as having CIA developed features of a systemic disease, most often GCA. CONCLUSION: The majority of patients (66%) with histopathologically proven aortitis have CIA at the time of surgery. CIA patients infrequently report new symptoms over time, but new vascular lesions requiring surgery commonly occur. Serial follow-up including large vessel imaging is strongly advised for all aortitis patients.

A subcutaneous fluid collection after the Bentall procedure in a female patient with giant cell arteritis.

We report a 61-year-old woman with a history of untreated inflammatory symptoms, who underwent a Bentall procedure for a rapidly evolving aneurysm. Postoperatively, she presented with a sub-sternal pulsatile mass communicating with a periaortic collection. After excluding surgical and infectious complications, an inflammatory seroma due to an uncontrolled vasculitis was suspected. We proposed a non-surgical approach involving immunosuppressive therapy.

Dural arteriovenous fistula mimicking temporal arteritis.

INTRODUCTION: Dural arteriovenous fistula (dAVF) is a very rare disease characterized by an abnormal vascular communication between arteries and veins in dural mater. It frequently presents with intracranial haemorrhage. Common presenting symptoms are headache and seizures. CASE REPORT: Here we report a case of dAVF in which the patient's symptoms mimic a temporal arteritis in a 23-year-old woman. She presented with painful mass at forehead for 9 months with frontotemporal headache. Magnetic resonance imaging demonstrated dural arteriovenous fistula. CONCLUSION: Since both diseases have different prognosis but similar presentation, it is important to ensure that there is no dural arteriovenous fistula in patient with suspected temporal arteritis.

A 59-year-old woman with visible precordial pulsations.

CLINICAL INTRODUCTION: A retired 59-year-old woman presented to the cardiology clinic concerned with cardiac pulsations that were visible on her chest wall. These were not associated with dyspnoea, syncope or chest discomfort.Of note, 8 years previously, she complained of recurrent nocturnal diaphoresis and 5 kg weight loss. Blood sampling at that time revealed a microcytic anaemia, reactive thrombocytosis and raised inflammatory markers (erythrocyte sedimentation rate 99 mm/hour, C-reactive protein 161 mg/L). Following an episode of transient diplopia, ophthalmoscopy demonstrated a cotton wool spot in the left inferotemporal retinal arcade. She commenced a 2-year tapering course of 1 mg/kg prednisolone.On examination, she had a lean physique with a supine blood pressure of 162/60 mm Hg and palpable Corrigan's pulse. She had a prominent apical pulsation and a loud early diastolic murmur was present at the left sternal edge radiating to the apex. Echocardiography showed severe central aortic regurgitation and a dilated aortic root (see online supplementary figure 1). Cardiac CT was performed to clarify the diagnosis (figure 1).DC1SP110.1136/heartjnl-2017-312193.supp1Supplementary file 1 heartjnl;104/3/273/F1F1F1Figure 1Contrast-enhanced CT of the thorax at index presentation (A) and 6 months (B). Prospective ECG-gated cardiac CT angiogram (75% R-R interval) performed at 8 years from index presentation (C) with a stretched multiplanar reconstruction of the aortic annulus, aortic root and thoracic aorta (D). QUESTION: Which of the following diagnoses best explains this presentation?Ankylosing spondylitisTakayasu arteritisSalmonellosisIgG4-related aortitisGiant cell aortitis.

[Ultrasound biomicroscopy in giant cell arteritis]. / Ultraschallbiomikroskopie bei Riesenzellarteriitis.

Giant cell arteritis may lead to irreversible blindness due to an anterior ischemic optic neuropathy or central retinal artery occlusion. Diagnosis of the giant cell arteritis is sometimes a problem: Difficulties may arise since a negative biopsy can not rule out the diagnosis. This case report demonstrates acute changes in the temporal artery as demonstrated by Ultrasound Biomicroscopy and documents its course during therapy with corticosteroids.

[Surgical and endoluminal management of the inflammatory aortitis: A Tunisian center experience]. / Traitement chirurgical et endovasculaire des atteintes aortiques inflammatoires : expérience d'un centre tunisien.

Non-infectious aortitis is usually due to giant-cell arteritis, Takayasu disease or Behçet disease. The main aortic lesions are stenoses, occlusions and aneurysms in the Takayasu disease and aneurysms in the Behçet disease and giant-cell arteritis. Treatment is based on corticosteroid therapy and surgery. Endoluminal management is now the rule. We report a retrospective descriptive study of 10 patients who underwent surgical or endoluminal management of inflammatory lesions of the aorta between January 2000 and December 2015. There were 4 cases of Takayasu disease and 6 cases of Behçet disease. The aortic lesions were aneurysmal in all of the patients with Behçet disease. In the patients with Takayasu disease, aortic occlusions predominated, associated with other arterial lesions. Four patients with Behçet disease were managed surgically, and 2 patients underwent endovascular repair. All of the patients with Takayasu disease underwent surgery. Two patients died in the postoperative period, and two patients died during long-term follow-up. Systematic screening, as well as regular monitoring of the entire aorta during the follow-up, is necessary due to the frequency of aortic aneurysms.

Fever of unknown origin, giant cell arteritis, and aortic dissection.

Giant cell arteritis is one of the most frequent causes of pyrexia of unknown origin after infectious or malignant causes have been ruled out. In this case report we describe a 66-year old female patient, who after five weeks of remitting fever developed a life-threatening, painless severe aortic dissection. The timely use of modern imaging technologies such as magnetic resonance angiography or positron emission computed tomography could in the future be of help to recognize aortic involvement early and to avoid this devastating complication in patients with fever of unknown origin.

Endovascular repair of inflammatory aortic aneuvrysm.

INTRODUCTION: While open repair was the gold standard treatment of atherosclerotic abdominal aortic aneurysms, its place in the treatment of inflammatory aortic aneurysms remains controversial. AIM: To specify the place of endovascular repair in the treatment of inflammatory aneurysms. OBSERVATION: We report 2 cases of inflammatory aortic aneurysms. The first patient was followed for Behçet's disease and the other patient for Giant Cell arteritis 's disease. We noted a biological inflammatory syndrome. CT scan suggested the diagnosis of inflammatory aortic aneurysm. We have implemented a bifurcated stent graft for the patient with a Behcet's disease. For the second patient, we converted to conventional surgery. Corticosteroid therapy was prescribed in two cases with favorable evolution. CONCLUSION: Endovascular repair is a good alternative to surgery for inflammatory aneurysms. Medical therapy occupies a prominent place in its therapeutic management.

Giant cell aortitis: clinical presentation and outcomes in 40 patients consecutively operated on.

OBJECTIVES: Giant cell arteritis (GCA) may affect mid-size and large-size arteries. Although temporal arteritis is a well-characterized clinical entity, GCA of the thoracic aorta remains ill defined. The aim of the study was to evaluate the clinical presentation, surgical and mid-term outcomes in patients operated for GCA of the thoracic aorta. METHODS: A retrospective review of patients operated for GCA of the thoracic aorta was conducted. The diagnosis of GCA was established by the pathology report. RESULTS: Forty consecutive patients (mean age of 66.6 ± 9.1 years) with a diagnosis of GCA of the thoracic aorta were operated on. A history of polymyalgia rheumatica or temporal arteritis was positive in 22.5% of patients. Emergency surgery was performed in 10% of patients (3 'type A' dissections and 1 'type B'). Mega-aorta syndrome was present in 10% of patients. Involvement of the ascending aorta was present in 100% of patients. One patient had a previous branched thoracic endovascular replacement (TEVAR) with a type I proximal endoleak. In 4 patients, the thoracic aorta was totally replaced. Eighty-five percent of patients had an arch replacement; 79.4% a hemiarch and 20.6% a full arch. The mean circulatory arrest time was 16.3 ± 12.3 min. Eighty percent of patients had an aortic valve procedure; aortic valve replacement was performed in 50% of them and Bentall-De Bono/valve sparing in 50%. Cerebrovascular accident occurred in 2.5% of patients. No patient died during hospitalization. The mean hospital stay was 8.7 ± 3.0 days. The mean postoperative follow-up time was 4.2 ± 2.3 years, with a mean of 4.2 ± 2.2 thoraco-abdominal computed tomographies (CTs)/patient. Four patients had late reinterventions: 2 were valve-related, 1 for a distal type I endoleak treated with a distal TEVAR extension and 1 type II open thoraco-abdominal replacement for disease progression. One distal type I TEVAR endoleak was treated medically. Aortic diameter progressions on CT (mm/year) were 0.7 ± 1.0 mm for the arch, 1.2 ± 2.0 mm for the isthmus, 1.1 ± 1.7 mm for the mid-descending, 0.7 ± 0.9 mm for the aortic hiatus, 0.5 ± 0.5 mm for the supra-renal aorta and 0.6 ± 0.6 mm for the infra-renal aorta. One patient who declined reoperation on the descending aorta died suddenly 3 years after her initial operation. The 5-year overall survival rate was 91%. CONCLUSIONS: GCA of the thoracic aorta may be suspected in less than 25% of patients preoperatively. Clinical presentation may be acute or chronic with localized or diffused aortic involvement but always involved the ascending aorta. Surgery may be performed with excellent outcomes. Follow-up imaging is mandatory to assess aortic progression.

[Aortitis and surgery]. / Prise en charge chirurgicale des maladies inflammatoires de l'aorte.

Non-infectious aortitis are usually due to giant cell arteritis (temporal arteritis), Takayasu and Behçet's diseases. Aortitis should be suspected in the presence of aortic wall thickening or of aneurysm or occlusion of the aorta and its branches in the absence of characteristic cardiovascular risk factors. Surgery is required in case of severe damage. But the quiescence of the inflammatory disease must be obtained before endovascular or surgical treatment to prevent complications such as anastomotic false aneurysm or stent thrombosis, especially common in this disease. The frequency of aortic aneurysms (in particular of ascending aorta) in giant cell arteritis encourages its systematic screening, as well as regular monitoring of the entire aorta during the follow-up. Behçet's and Takayasu diseases require the greatest control of inflammation and the knowledge of some surgical tricks to avoid the risk of recurrence. The literature review shows that endovascular treatment could reduce perioperative morbidity but did not allow reducing long-term complications.

Methotrexate in refractory bilateral juvenile temporal arteritis: Report of a case.

Juvenile temporal arteritis is a rare inflammatory disease of the temporal arteries that affects young adults. The clinical course is benign and the surgical excision of the affected artery is usually curative. Here we report a case of bilateral juvenile temporal arteritis with significant peripheral eosinophilia and elevated IgE, refractory to surgical excision and even to a short course of corticosteroids. Methotrexate, added as a steroid-sparing agent, resulted in a good disease control.