Infectious aortitis and managing it at a community military hospital.

Infectious aortitis is a rare disease process which can be of fungal, viral or bacterial aetiology. This disease process is often incidentally found during concomitant infectious processes, likely due to haematogenous spread. Common sources are from cardiac, genitourinary and gastroenterologic sources. CT imaging of the aorta is essential in identifying physiological changes-wall thickness changes, ectasia and stenosis. We present a case of a female in her early 60s with a medical history of cardiomyopathy with heart failure and reduced ejection fraction, who was initially admitted for acute cholecystitis complicated by the development of gallstone pancreatitis. Imaging evaluation incidentally noted findings consistent with aortitis with a penetrating ulcer, and blood cultures were positive for Staphylococcus aureus bacteraemia, confirming her diagnosis of infectious aortitis. She was started on intravenous antibiotics, required preoperative nutritional optimisation, and subsequently underwent an open aortic resection and aortoiliac reconstruction with rifampin-soaked Dacron graft.

Aortitis after administration of pegfilgrastim to a healthy donor for peripheral blood stem cell collection.

A 45-year-old man who was a sibling donor for allogeneic peripheral blood stem cell transplantation (allo-PBSCT) was administered 7.2 mg of pegfilgrastim for stem cell collection. Peripheral blood stem cells were collected 4 days after administration of pegfilgrastim (Day 4) and 4.32 × 106 /kg of CD34-positive cells per recipient body weight were obtained. Fever of 38 ℃ or higher and left submandibular pain appeared on Day 6. Ultrasonography and contrast-enhanced computed tomography (CT) showed wall thickening of the carotid artery and the abdominal aorta. We carefully excluded the possibilities of cardiovascular and autoimmune diseases by thorough examination, and ultimately diagnosed pegfilgrastim-induced aortitis. The patient's fever resolved rapidly after treatment with prednisolone (PSL) 1 mg/kg. We began to taper PSL after eight days. Sixty-one days after starting PSL, we confirmed that abdominal aortic wall thickening had improved by contrast-enhanced CT. We continued to taper off PSL and stopped 141 days later with no relapse thereafter. This is the first case report of pegfilgrastim-induced aortitis in an allo-PBSCT donor. Careful monitoring is warranted when administering pegfilgrastim to donors even without past medical history.

Recurring episodes of bundle branch reentry ventricular tachycardia due to aortitis preceded by SARS-CoV-2 infection: a case report.

BACKGROUND: SARS-CoV-2 may trigger both vasculitis and arrhythmias as part of a multisystem inflammatory syndrome described in children as well as in adults following COVID-19 infection with only minor respiratory symptoms. The syndrome denotes a severe dysfunction of one or more extra-pulmonary organ systems, with symptom onset approximately 2-5 weeks after the COVID-19 infection. In the present case, a seemingly intractable ventricular tachycardia preceded by SARS-CoV2 infection was only managed following the diagnosis and management of aortitis. CASE PRESENTATION: A 69-year-old woman was hospitalized due to syncope, following a mild COVID-19 infection. She presented with paroxysmal atrial fibrillation and intermittent ventricular tachycardia interpreted as a septum-triggered bundle branch reentry ventricular tachycardia, unaffected by amiodaron, lidocaine and adenosine. A CT-scan revealed inflammation of the aortic arch, extending into the aortic root. In the following days, the tachycardia progressed to ventricular storm with intermittent third-degree AV block. A temporary pacemaker was implanted, and radiofrequency ablation was performed to both sides of the ventricular septum after which the ventricular tachycardia was non-inducible. Following supplemental prednisolone treatment, cardiac symptoms and arrythmia subsided, but recurred after tapering. Long-term prednisolone treatment was therefore initiated with no relapse in the following 14 months. CONCLUSION: We present a rare case of aortitis complicated with life-threatening ventricular tachycardia presided by Covid-19 infection without major respiratory symptoms. Given a known normal AV conduction prior to the COVID-19 infection, it seems likely that the ensuing aortitis in turn affected the septal myocardium, enabling the reentry tachycardia. Generally, bundle branch reentry tachycardia is best treated with radiofrequency ablation, but if it is due to aortitis with myocardial affection, long-term anti-inflammatory treatment is mandatory to prevent relapse and assure arrhythmia control. Our case highlights importance to recognize the existence of the multisystem inflammatory syndrome in adults (MIS-A) following COVID-19 infection in patients with alarming cardiovascular symptoms. The case shows that the early use of an CT-scan was crucial for both proper diagnosis and treatment option.

Multidisciplinary diagnosis and management of inflammatory aortic aneurysms.

BACKGROUND: Inflammatory abdominal aortic aneurysms (IAAAs) are a variant involving a distinct immunoinflammatory process, with nearly one half believed to be associated with IgG4-related disease (IgG4-RD). METHODS: MEDLINE and Google Scholar searches were conducted for English-language publications relevant to inflammatory aortic aneurysms from January 1970 onward. The search terms included inflammatory aortic aneurysms, aortitis, periaortitis, IgG4-related disease, and retroperitoneal fibrosis. Relevant studies were selected for review based on their relevance. RESULTS: Morphologically, IAAAs are characterized by a thickened aneurysm wall often displaying contrast enhancement and elevated metabolic activity on fluorine-18 fluorodeoxyglucose-positron emission tomography imaging. A strong association exists with perianeurysmal and retroperitoneal fibrosis. Although the rupture risk appears lower with IAAAs than with noninflammatory abdominal aortic aneurysms (AAAs), the currently recommended diameter threshold for operative management is the same. Open repair has been associated with increased morbidity compared with noninflammatory AAAs, and a retroperitoneal approach or minimal dissection transperitoneal approach has been recommended to avoid duodenal and retroperitoneal structural injuries. Endovascular aneurysm repair has been increasingly used, especially for patients unfit for open surgery. It is important to exclude an infectious etiology before the initiation of immunosuppressive therapy or operative repair. Multimodality imaging follow-up is critical to monitor disease activity and secondary involvement of retroperitoneal structures by the associated fibrotic process. Maintenance of immunosuppressive therapy will be needed postoperatively for most patients with active systemic disease, especially those with IgG4-RD and those with persistent symptoms. Additional interventions aimed at ureteral decompression could also be required, and lifelong follow-up is mandatory. CONCLUSIONS: Preoperative multimodality imaging is a diagnostic cornerstone for assessment of the disease extent and activity. IgG4-RD is an increasingly recognized category of IAAAs, with implications for tailoring adjunctive medical therapy. Open surgical repair remains the procedure of choice, although endovascular aneurysm repair is increasingly being offered. Maintenance immunosuppressive therapy can be offered according to the disease activity as assessed by follow-up imaging studies.

Inflammatory Diseases of the Aorta: JACC Focus Seminar, Part 2.

Inflammatory aortitis is most often caused by large vessel vasculitis (LVV), including giant cell arteritis, Takayasu's arteritis, immunoglobulin G4-related aortitis, and isolated aortitis. There are distinct differences in the clinical presentation, imaging findings, and natural history of LVV that are important for the cardiovascular provider to know. If possible, histopathologic specimens should be obtained to aide in accurate diagnosis and management of LVV. In most cases, corticosteroids are utilized in the acute phase, with the addition of steroid-sparing agents to achieve disease remission while sparing corticosteroid toxic effects. Endovascular and surgical procedures have been described with success but should be delayed until disease control is achieved whenever possible. Long-term management should include regular follow-up with rheumatology and surveillance imaging for sequelae of LVV.

Aortitis which developed after the administration of granulocyte-colony stimulating factor.

Drug-induced aortitis is rare; thus, the diagnosis of drug-induced aortitis could be delayed unless clinicians are aware of the disease entity. Herein, we describe the case of a 66-year-old woman who developed aortitis after administration of granulocyte-colony stimulating factor (G-CSF) during chemotherapy for her breast cancer. Thickening of the aortic wall was clearly detected by computed tomography (CT) and magnetic resonance imaging. After excluding the other possible aetiologies, an association between G-CSF and the development of aortitis was highly suspected. Corticosteroid treatment rapidly regressed the aortitis, as confirmed by follow-up CT examination. G-CSF analog is generally well tolerated; however, there are limited case reports of G-CSF-associated aortitis, suggesting the causative effect of G-CSF in the development of aortitis. Currently, G-CSF-associated aortitis has received little attention among rheumatologists. As the delayed diagnosis results in irreversible changes in the aorta, not only oncologists but also rheumatologists should be aware of this unrecognized disease entity, G-CSF-associated aortitis.

A strobe multicenter descriptive study of 55 infectious aortitis.

Infectious aortitis (IA) is a rare and severe disease. The treatment classically associates open surgery with prolonged antibiotic therapy. This study aimed to describe clinical characteristics, medical and surgical supports in a large and current series of IA.We conducted a retrospective multicenter study of native aorta IA, between 2000 and 2019. Inclusion criteria were the presence of a microorganism on blood culture, aortic sample or any other validated technique and structural anomaly in imaging.We included 55 patients (85% men), with a median age of 65. Microbiology data substantially differed from previous studies with 12 Gram-negative rods IA, of which only 3 due to Salmonella spp., 24 Gram-positive cocci IA of which 12 Streptococcus spp., and 18 IA due to intracellular growth and/or fastidious microorganisms, of which 8 Coxiella burnetii, 3 Treponema pallidum, and 5 tuberculosis suspicious cases. Fifteen patients (27%) presented with thoracic IA, 31 (56%) with abdominal IA, and 9 (16%) with thoraco-abdominal IA. Eight patients had no surgery, 41 underwent open surgery, only 4 endovascular aneurysm repair, and 2 a combination of these 2 techniques. Nine patients died before 1-month follow-up. There was no difference in the mortality rate between the different types of germ or localization of IA.The variety of germs involved in IA increases. Positron emission tomography-computed tomography scan is a very useful tool for diagnosis. Surgery is still mainly done in open approach and a prospective multicenter study seems necessary to better determine the place of endovascular aneurysm repair versus open surgery.

Aortitis in the setting of catastrophic antiphospholipid syndrome in a patient with systemic lupus erythematosus.

Catastrophic antiphospholipid syndrome (CAPS) is a rare condition characterized by multiple thromboses affecting mainly small vessels in a short period of time in patients with antiphospholipid antibodies. A high suspicion index is mandatory in order to initiate rapidly aggressive immunomodulatory therapy to avoid a very poor prognosis. Systemic lupus erythematosus (SLE) is often associated with antiphospholipid syndrome, with a worse outcome when the catastrophic features occur. We report the case of a 64-year-old woman with a clinical debut of SLE who presented concomitantly with CAPS with several thrombosis affecting the kidney, spleen and bilateral limbs with blue toe syndrome in both legs. Furthermore, she presented with aortitis, with a malaise and myalgias and general syndrome (asthenia, hyporexia and mild weight loss). Fortunately, she had a good response to multi-target combination therapy (anticoagulants, corticosteroids, hydroxychloroquine, intravenous immunoglobulins, plasma exchange and rituximab). Here, we discuss the association between aortitis and CAPS secondary to SLE, and review the literature regarding similar conditions.

Saprochaete capitata aortitis in an immunocomopetent patient after myocardial revascularization.

Saprochaete species infection is a rare fungal disease reported so far only in immunocompromised patients. We describe the first case of aortitis caused by Saprochaete capitata, presenting as ascending aorta aneurysm, with secondary endophthalmitis in an immunocompetent patient. Infection by Saprochaete capitata is potentially fatal, with a mortality ranging from 50% to 90% of cases. In the present case aortic aneurysm caused by Saprochaete capitata aortitis was successfully treated by the combination of accurate diagnosis with surgical and specific antifungal therapy.

Mechanism and biomarkers in aortitis--a review.

Aortitis can be the manifestation of an underlying infectious or noninfectious disease process. An autoimmune cause is suggested in a large proportion of noninfectious causes. Similar to other autoimmune diseases, the pathophysiology of aortitis has been investigated in detail, but the etiology remains unknown. Most cases of aortitis often go undetected for a long time and are often identified at late stages of the disease. Recent advances in imaging techniques have significantly improved the diagnosis of aortitis. However, significant challenges associated with the imaging techniques limit their use. Several routine inflammation-based markers, such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and inflammatory cytokines, are nonspecific and, therefore, have limited use in the diagnosis of aortitis. The search for more specific serum biomarkers, which can facilitate detection and progression is under progress. Several autoantibodies have been identified, but assigning their role in the pathogenesis as well as their specificity remains a challenge. The current review addresses some of these issues in detail. KEY MESSAGES: • Noninfectious aortitis is an autoimmune disease. • Several biomarkers, including cytokines and autoantibodies, are increased in aortitis. • Imaging techniques, commonly used to detect aortitis, are associated with the high cost and technical challenges. • There is a need to develop low-cost biomarker-based detection tools. • The knowledge of biomarkers in aortitis detection is discussed.

Clostridium septicum aortitis: A kiss of the devil.

BACKGROUND: Clostridium septicum aortitis is a lethal infection. C. septicum has a strong association with an underlying malignancy, most commonly in the colon. AIM: Early identification methods and management strategies of C. Septicum infection. MATERIALS AND METHODS: We present a 64-year-old man with aortic aneurysm and C. septicum bacteremia with unknown malignancy who passed away on the fourth day of hospitalization despite emergent endovascular intervention. Computed tomography showed periaortic gas which is the hallmark of infection. DISCUSSION: This case report highlights the need of prompt surgical treatment and its different modalities along with the early use of appropriate antibiotics due to the rapid spread of infection associated with high fatality. The authors also discuss the association of C. septicum aortitis with underlying occult malignancies. CONCLUSION: Delay in identification and treatment of C. Septicum is associated with very high mortality rates.

Aspergillus aortitis & aortic valve endocarditis after coronary surgery.

The authors report a case of Aspergillus aortitis and aortic valve endocarditis that developed after coronary artery bypass surgery resulting in recurrent, embolic, bilateral lower extremity ischemia. This necessitated multiple lower extremity embolectomies followed by redo-sternotomy, extensive annular debridement, root reconstruction with a modified Bentall technique, and hemiarch replacement. This case highlights the challenges in diagnosis and management of this rare disease entity and some of its devastating complications.

Successful Treatment of an Aortotomy Site Aspergillus Infection.

Aspergillus aortitis is a rare, often fatal infection. Here, we report successful treatment of an aortotomy site that became infected by Aspergillus spp. after valve replacement. Surgical debridement, combined with antifungal therapy, allowed a favorable outcome.

Imaging aortic wall inflammation.

Inflammation affects the aortic wall through complex pathways that alter its biomechanical structure and cellular composition. Inflammatory processes that predominantly affect the intima cause occlusive disease whereas medial inflammation and degeneration cause aneurysm formation. Aortic inflammatory pathways share common metabolic features that can be localized by smart contrast agents and radiolabelled positron emission tomography (PET) tracers. 18F-Fluorodeoxyglucose (18F-FDG) is a non-specific marker of metabolism and has been widely used to study aortic inflammation in various diseased aortic states. Although useful in detecting disease, 18F-FDG has yet to demonstrate a reliable link between vessel wall disease and clinical progression. 18F-Sodium fluoride (18F-NaF) is a promising biological tracer that detects microcalcification related to active disease and cellular necrosis within the vessel wall. 18F-NaF shows a high affinity to bind to diseased arterial tissue irrespective of the underlying inflammatory process. In abdominal aortic aneurysms, 18F-NaF PET/CT predicts increased rates of growth and important clinical end-points, such as rupture or the requirement for repair. Much work remains to be done to bridge the gap between detecting aortic inflammation in at-risk individuals and predicting adverse clinical events. Novel radiotracers may hold the key to improve our understanding of vessel wall biology and how this relates to patients. Combined with established clinical and morphological assessment techniques, PET imaging promises to improve disease detection and clinical risk stratification.

A case of Brucella aortitis associated with development of thoracic aortic aneurysm and aortobronchial fistula.

OBJECTIVES: The purpose of this case report is to document the occurrence of granulomatous aortitis complicated by formation of a saccular aneurysm and aortobronchial fistula due to Brucella infection. METHODS: A 65-year-old man with a history of feral swine hunting presented with hemoptysis and was found to have a saccular thoracic aortic aneurysm and associated aortobronchial fistula. The aneurysm underwent operative repair with closure of the aortobronchial fistula. RESULTS: Histopathological examination of the aneurysm wall revealed evidence of granulomatous aortitis. Cultures of the blood and aortic wall tissue were positive for Brucella suis. CONCLUSIONS: Although rare, Brucella infection should be considered in the differential diagnosis of aortic aneurysm with granulomatous aortitis.

Incidence of Aortitis in Surgical Specimens of the Ascending Aorta Clinical Implications at Follow-Up.

The objectives of this study are to evaluate the incidence of aortitis on a surgical population, establish any relationship with systemic diseases, verify early and late surgical results and provide clinical and radiological follow-up to determine factors potentially predicting progression of the disease and influencing late outcome. From 2009 to 2017, 237 patients underwent elective operations on the ascending aorta. Segments of the excised tissues were routinely sent for histologic evaluation, providing adequate data in 178 (75%) for a clinical and pathologic correlation. Patients with aortitis (Group 1) (n = 26) were compared with 152 with atherosclerotic or degenerative disease (Group 2). Incidence of aortitis was 15%, being clinically isolated in 73%. In 24 patients (92%), a giant cell aortitis was found. Actuarial survival at 3 years is 88% in Group 1 and 98% in Group 2 and 74% and 98% at 5 years, respectively (P = 0.016). A control angio-computed tomography revealed an increased descending aorta diameter in 2 out of 14 late survivors. A positron emission tomography showed presence of arteritis in other vascular segments in 3 patients. Clinically isolated aortitis is extremely frequent in patients with inflammatory aortic disease. The diagnosis is often difficult and may be supported by routine pathologic evaluation of surgical explants and by multimodality imaging. The latter should be employed to allow adequate patient follow-up and to disclose potential recurrences in untreated aortic segments.