Analysis of clinical features and prognostic factors in Takayasu arteritis involving pulmonary hypertension: A retrospective study.

BACKGROUND: Multiple takayasu arteritis (TA) is a chronic nonspecific large to medium vasculitis disease that mainly accumulates the aorta and its branches. Pulmonary vascular disease is often seen as stenosis and occlusion, and patients may show no moderate to severe pulmonary hypertension (PH). This study aims to summarize the clinical characteristics and analysis of prognostic factors in patients with PH caused by TA. METHODS: Patients diagnosed with aortitis involving the pulmonary artery by pulmonary arteriography or pulmonary artery and total aortic computed tomography arteriography (CTA). All patients underwent detailed clinical assessment, laboratory data collection, and analysis of imaging data. Patients were followed up and factors affecting the prognosis of the pulmonary arteries were analyzed. RESULTS: Most of the patients' complaints were chest tightness, shortness of breath, decreased activity tolerance, hemoptysis and chest pain. 56.90% of the patients were in at the time of admission. Echocardiographic estimation of pulmonary artery systolic pressure was 90.39 ±â€…22.87 mm Hg. In terms of laboratory tests, 39.66%% of the patients had elevated C-reactive protein and erythrocyte sedimentation rate, and amino-terminal natriuretic peptide precursor on admission. In terms of imaging, all patients had pulmonary artery involvement, which was combined with aortic involvement in 31.03%. Nuclide lung perfusion/ventilation imaging of the patients revealed multiple perfusion defects/absences in the segmental and subsegmental distribution of the lungs. Univariate Cox regression model analysis suggested that patients' WHO functional class at admission, age ≧ 51 years at the time of consultation, and amino-terminal natriuretic peptide precursor ≧ 3500 pg/mL were factors affecting the prognosis. Further multifactorial Cox regression model analysis suggested amino-terminal natriuretic peptide precursor ≧ 3500 pg/mL was an independent predictor of poor prognosis with a hazard ratio (HR) value of 5.248. CONCLUSION: Electrocardiogram and echocardiogram may suggest an increased right heart load; some patients have elevated serum inflammatory indexes. Characteristic imaging manifestations include widening of the main pulmonary artery, multiple pulmonary segmental and subsegmental stenoses.

Pregnancy in Takayasu's arteritis has a high risk of hypertension-related fetomaternal complications: A retrospective study of a Turkish cohort.

BACKGROUND: This study aimed to examine fetomaternal outcomes in pregnant women in a large Turkish Takayasu arteritis (TAK) cohort and to evaluate the effects of pregnancy on the disease in those patients. METHODS: This is a cohort study involving 296 pregnancies of 112 TAK patients from 8 tertiary rheumatology centers in Turkey. Pregnancies were divided into 2 groups as pre-d (before disease onset) and post-d (after disease onset). In addition, post-d pregnancies were further divided into 2 subgroups according to fetomaternal complications (FMC) development status. Finally, patients were grouped into those with and without a history of pregnancy after disease onset. RESULTS: In post-d pregnancies, rates of worsening hypertension, new-onset hypertension, and preeclampsia were higher than in pre-d pregnancies (0.9% vs 16%, P < .001, 0.5% vs 5.3%, P = .012, and 0% vs 4%, P = .013, respectively). Patients with FMC were more likely to have renal artery involvement (65% vs 21%, P = .003). The patients who had post-d were younger, had longer disease duration, and had more relapses number than other patients (P < .001, P = .028, P = .016, respectively). Vasculitis Damage Index (VDI) results were similar in patients with or without post-d pregnancies. CONCLUSION: Pregnancies after disease onset were found to be associated with HT and preeclampsia/eclampsia. HT-related FMCs are increased in TAK, and patients with renal artery involvement are at higher risk. The number of relapses increases in patients who become pregnant after disease onset, but pregnancy was not an independent risk factor for relapse. Pregnancy after the onset of disease had no negative effect on VDI.

Refractory convulsive syncope in pregnancy: a rare presentation of Takayasu's arteritis - a case report and literature review.

BACKGROUND: Neurological manifestation of Takayasu's Arteritis (TA) in pregnancy presenting as convulsive syncope is extremely rare, and poses a serious diagnostic dilemma due to other vast causes of fits in pregnancy. OBJECTIVE: We aimed to present and shed more light on a case of TA with convulsive syncope in pregnancy refractory to anticonvulsants for seven weeks, and review the literature on the management of TA in pregnancy. CASE PRESENTATION: A gravida 4 para 3+0 at 28 weeks of amenorrhea presented with repeated episodes of the sudden loss of consciousness, followed by a fall and jerking of the limbs. These were refractory to anticonvulsants that she had used for seven weeks. Physical examination revealed undetectable pulse and blood pressure (BP) in the upper limbs but elevated BP in the lower limbs. Further investigations confirmed TA and she improved on steroids and antihypertensives. CONCLUSION: This case typically describes the unexpected presentation of TA with convulsive syncope. It calls for meticulous clinical assessment of epileptic seizures in pregnancy to avoid a late diagnosis of TA and its potential poor outcomes.

Acute myocardial infarction with left main coronary artery ostial negative remodelling as the first manifestation of Takayasu arteritis: a case report.

BACKGROUND: Takayasu arteritis is a chronic inflammatory disease involving the aorta and its major branches. Acute myocardial infarction rarely but not so much presents in patients with Takayasu arteritis, and the preferable revascularization strategy is still under debate. CASE PRESENTATION: A 22-year-old female with Takayasu arteritis presented with acute myocardial infarction. Coronary angiography and intravenous ultrasound (IVUS) showed that the right coronary artery (RCA) was occluded and that there was severe negative remodelling at the ostium of the left main coronary artery (LMCA). The patient was treated by primary percutaneous transluminal coronary angioplasty (PTCA) with a scoring balloon in the LMCA, without stent implantation. After 3 months of immunosuppressive medication, the patient received RCA revascularization by stenting. There was progressive external elastic membrane (EEM) enlargement of the LMCA ostium demonstrated by IVUS at 3 and 15 months post-initial PTCA. CONCLUSION: Here, we report a case of Takayasu arteritis with involvement of the coronary artery ostium. Through PTCA and long-term immunosuppressive medication, we found that coronary negative remodelling might be reversible in patients with Takayasu arteritis.

Poor physical activity levels and cardiorespiratory fitness among patients with childhood-onset takayasu arteritis in remission: a cross-sectional, multicenter study.

BACKGROUND: It is currently unknown whether patients with childhood-onset Takayasu disease (c-TA) are prone to physical inactivity and poor aerobic capacity. In this study, we assessed physical activity levels and cardiorespiratory fitness along with health-related quality of life (HRQL) and various traditional and non-traditional risk factors in patients with c-TA vs. healthy controls. METHODS: c-TA patients with non-active disease (n = 17) and age- and sex-matched healthy controls (n = 17) were enrolled in the study. We assessed physical activity levels, aerobic capacity, body composition, systemic inflammation, cardiometabolic markers, disease-related parameters, and HRQL. RESULTS: c-TA patients showed greater time spent in sedentary behavior (P = 0.010), and lower moderate-to-vigorous physical activity (P > 0.001) and lower step counts per day (P > 0.001). VO2peak (P < 0.001) and chronotropic response (P = 0.016) were significantly lower in patients with c-TA and they had worse HRQL in physical domain (P < 0.001), lower bone mineral content and density, and higher insulin levels vs. healthy controls (all P ≤ 0.05). CONCLUSIONS: c-TA patients exhibited reduced physical activity levels and aerobic capacity, worse cardiometabolic risk factors and HRQL parameter compared with healthy peers. Physical inactivity and aerobic deconditioning emerge as potentially novel risk factors for c-TA. The role of physical activity interventions in preventing poor outcomes and improving HRQL in c-TA remains to be explored.

Increased modifiable cardiovascular risk factors in patients with Takayasu arteritis: a multicenter cross-sectional study.

BACKGROUND: Modifiable cardiovascular risk factors (MCRFs), such as those related to aerobic capacity, muscle strength, physical activity, and body composition, have been poorly studied in Takayasu arteritis (TAK). Therefore, the aim of the study was to investigate MCRFs and their relationships with disease status and comorbidities among patients with TAK. METHODS: A multicenter cross-sectional study was conducted between 2019 and 2020, in which 20 adult women with TAK were compared with 16 healthy controls matched by gender, age, and body mass index. The following parameters were analyzed: aerobic capacity by cardiopulmonary test; muscle function by timed-stands test, timed up-and-go test, and handgrip test; muscle strength by one-repetition maximum test and handgrip test; body composition by densitometry; physical activity and metabolic equivalent by IPAQ, quality of life by HAQ and SF-36; disease activity by ITAS2010 and NIH score; and presence of comorbidities. RESULTS: Patients with TAK had a mean age of 41.5 (38.0-46.3) years, disease duration of 16.0 (9.5-20.0) years, and a mean BMI of 27.7±4.5 kg/m2. Three out of the 20 patients with TAK had active disease. Regarding comorbidities, 16 patients had systemic arterial hypertension, 11 had dyslipidemia, and two had type 2 diabetes mellitus, while the control group had no comorbidities. TAK had a significant reduction in aerobic capacity (absolute and relative VO2 peak), muscle strength in the lower limbs, increased visceral adipose tissue, waist-to-hip ratio, reduced walking capacity, decreased weekly metabolic equivalent, and quality of life (P< 0.05) as compared to controls. However, there were no correlations between these MCRFs parameters and disease activity. CONCLUSIONS: TAK show impairment in MCRFs; therefore, strategies able to improve MCRF should be considered in this disease.

Takayasu's Arteritis from the Patients' Perspectives: Measuring the Pulse to the Patient-Reported Outcomes.

BACKGROUND: Patients with Takayasu's arteritis (TA) experience important changes in lifestyle, quality of life, and functional status due to ischemic symptoms or treatment toxicity. PURPOSE: To describe the clinical characteristics and the patient-reported outcomes (PROs), such as quality of life, disability, fatigue, and perception/impact of the disease in Mexican patients with TA. METHODS: Cross-sectional study including patients with established diagnosis of TA recruited at a tertiary care center. Demographics, comorbidities, clinical characteristics, laboratory, imaging, and treatment were retrieved. Disease activity (the Indian Takayasu Clinical Activity Score (ITAS) 2010), damage (Vasculitis Damage Index (VDI)), quality of life (Short Form 36 (SF-36)), disability (Health Assessment Questionnaire Disability Index (HAQ-DI)), fatigue (Multidimensional Fatigue Inventory-20), and patient's disease perceptions were assessed. RESULTS: Fifteen women were included, with a median age of 41 years (interquartile range (IQR) 30-45) and disease duration of 108 months (IQR 55-197). Median ITAS 2010 and VDI scores were 0 (IQR 0-2) and 3 points (IQR 2-6), respectively. Mean SF-36 score was 71.38 ± 13.39, with mean physical and mental component summaries of 66.52 ± 13.37 and 76.24 ± 14.89, respectively. HAQ-DI mean score was 0.48 ± 0.62, being grip the most affected domain. Among fatigue subscales, the higher scores were present in the physical fatigue (16.3 ± 5.8). Correlations between the HAQ-DI and the VDI score (r = 0.64, P = 0.03); between the general fatigue, score, and disease duration (r = -0.71, P = 0.01); and between the SF-36 total score and the HAQ-DI (r = -0.87, P = 0.0004) were found. CONCLUSIONS: It is important to identify disease-specific outcomes of interest to the patients to develop tools that assess them with a holistic approach.

Increased modifiable cardiovascular risk factors in patients with Takayasu arteritis: a multicenter cross-sectional study

Abstract Background: Modifiable cardiovascular risk factors (MCRFs), such as those related to aerobic capacity, muscle strength, physical activity, and body composition, have been poorly studied in Takayasu arteritis (TAK). Therefore, the aim of the study was to investigate MCRFs and their relationships with disease status and comorbidities among patients with TAK. Methods: A multicenter cross-sectional study was conducted between 2019 and 2020, in which 20 adult women with TAK were compared with 16 healthy controls matched by gender, age, and body mass index. The following parameters were analyzed: aerobic capacity by cardiopulmonary test; muscle function by timed-stands test, timed up-and-go test, and handgrip test; muscle strength by one-repetition maximum test and handgrip test; body composition by densitometry; physical activity and metabolic equivalent by IPAQ, quality of life by HAQ and SF-36; disease activity by ITAS2010 and NIH score; and presence of comorbidities. Results: Patients with TAK had a mean age of 41.5 (38.0-46.3) years, disease duration of 16.0 (9.5-20.0) years, and a mean BMI of 27.7±4.5 kg/m2. Three out of the 20 patients with TAK had active disease. Regarding comorbidities, 16 patients had systemic arterial hypertension, 11 had dyslipidemia, and two had type 2 diabetes mellitus, while the control group had no comorbidities. TAK had a significant reduction in aerobic capacity (absolute and relative VO2 peak), muscle strength in the lower limbs, increased visceral adipose tissue, waist-to-hip ratio, reduced walking capacity, decreased weekly metabolic equivalent, and quality of life (P< 0.05) as compared to controls. However, there were no correlations between these MCRFs parameters and disease activity. Conclusions: TAK show impairment in MCRFs; therefore, strategies able to improve MCRF should be considered in this disease.(AU)

Assessment of damage in Takayasu's arteritis.

OBJECTIVE: To assess the progression and the factors associated with damage in Takayasu's arteritis (TAK) patients during routine follow-up. METHODS: Patients diagnosed with TAK and had >6 months follow-up were enrolled in this study retrospectively. Takayasu's arteritis damage score (TADS) and vasculitis damage index (VDI) were determined at diagnosis and at the end of the follow-up and variables associated with damage scores were assessed. RESULTS: One-hundred fourteen patients (F/M: 101/13) were included in the study. The mean age at diagnosis, median symptom duration at baseline visit and mean follow-up duration were 35.3±13.3 years, 12 (0-360) months and 76.9±51.4 months, respectively. Median VDI score was 4.0 (1-8) and median TADS score was 7.0 (1-15) at baseline assessment. At the end of the follow-up, median VDI score increased to 5.0 (1-17) and TADS score to 8.0 (1-19). The median number of disease-related items were higher in TADS (8 items vs 4 items). At least one new corticosteroid (CS)-related damage item occurred in 35 patients (31%). Age at symptom-onset and cumulative CS doses were predictor factors for higher VDI score (≥5), whereas age at symptom-onset and disease duration were associated with increase in TADS (≥8). Gender and number of relapses were not associated with damage scores. CONCLUSION: Damage assessment with VDI seems to capture treatment-related damage better, whereas TADS provides some additional information on disease-related damage in Takayasu's arteritis. Older age at symptom onset, disease duration and cumulative CS dose were associated with higher damage scores. The relapse frequency did not influence the damage level in our routine-follow-up of TAK patients.

Patterns of clinical presentation in Takayasu's arteritis.

OBJECTIVE: Takayasu's arteritis (TAK) is a clinically heterogenous disease. Patterns of clinical presentation in TAK at diagnosis have not been well described, and a "triphasic pattern" of constitutional symptoms evolving into vascular inflammation and fibrosis has been reported but never systematically evaluated. METHODS: Patients with TAK were prospectively recruited from the National Institutes of Health (NIH) and the Vasculitis Clinical Research Consortium (VCRC). Based on clinical presentation at diagnosis, patients were divided into five categories: (1) constitutional symptoms alone, (2) carotidynia, (3) other vascular-associated symptoms, (4) major ischemic event, or (5) asymptomatic. Associated clinical characteristics were evaluated in each category. Preceding symptoms were also assessed to determine the presence of a triphasic disease pattern. RESULTS: A total of 275 patients with TAK were included (VCRC=208; NIH=67). Similar heterogeneity of clinical presentation was identified in each cohort: constitutional symptoms (8%), carotidynia (13-15%), other vascular symptoms (43-47%), major ischemic event (28-30%), and asymptomatic (2-6%). An increased relative proportion of males was seen in patients who presented with constitutional symptoms or were asymptomatic at diagnosis (p<0.01). Patients who presented with constitutional symptoms and major ischemic events were youngest at diagnosis. Patients in the asymptomatic group were oldest at diagnosis and often were not treated (p<0.01). Relapse was most frequent in patients who presented with carotidynia (p<0.01). A minority of patients (19%) who presented with a major ischemic event reported a triphasic pattern of disease. CONCLUSION: There are diverse clinical presentations at diagnosis in TAK. Patients do not necessarily progress sequentially through phases of disease.

Spectrum of paediatric rheumatic disorders at a tertiary hospital in Tanzania.

BACKGROUND: Paediatric rheumatic disorders are common in children and result in significant impairment in quality of life, morbidity and mortality. There is limited information on the burden of these disorders in lower income countries especially in sub-Saharan Africa. Few case reports have documented presence of paediatric rheumatic disorders in Tanzania. This study was conducted to determine the spectrum of rheumatic disorders among children at Muhimbili National Hospital (MNH). METHODS: This was a retrospective study conducted among children who were attended at MNH between January 2012 and August 2019. Paediatric patients seen in the out-patient clinics and those admitted in the wards were eligible. All patients with diagnosis of rheumatic disorders were identified from admission books and outpatient clinic logbooks, and later data were collected from their case notes and were recorded in clinical research forms. Collected information included age, sex, clinical features and laboratory tests results. RESULTS: A total of 52 children with mean age of 9.5 ± 4.3 years, 12 (40.4%) participants were aged above 10 years and 32 (61.5%) were females. Frequently reported clinical presentations were joint pain 44 (84.6%), joint swelling 34 (65.4%), fever 24 (46.2%) and skin rashes 21(40.4%). Juvenile idiopathic arthritis (JIA) was the predominant diagnosis reported in 28 (53.8%) participants followed by juvenile systemic lupus erythematosus 8 (15.4%), mixed connective tissue diseases 4 (7.7%) and juvenile dermatomyositis 4 (7.7%). Antinuclear antibody test was performed in 16 participants it was positive in 9 (56.2%). Nine participants were tested for anti-double stranded DNA test and 5 (55.6%) were positive for this test. C-reactive protein was tested in 46 participants out of which 32 (69.6%) had elevated levels. HIV was tested in 24 (46.2%) participants and results were negative. Thirty-five out of 52 (67.3%) participants had anaemia. Predominant drugs used for treatment of JIA include prednisolone and methotrexate. CONCLUSIONS: Paediatric rheumatic disorders are not uncommon in Tanzania-and were noted to affect more female children in this study. Predominant conditions included juvenile idiopathic arthritis (JIA), juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM).

A retrospective cohort study to assess PET-CT findings and clinical outcomes in Takayasu arteritis: does 18F-fluorodeoxyglucose uptake in arteries predict relapses?

The objective of this study is to investigate the use of PET-CT scan with 18F-fluorodeoxyglucose (18F-FDG) as a method to predict outcomes in patients with Takayasu arteritis (TAK), as well as to analyze associations between 18F-FDG PET-CT findings with disease relapses, sustained remission, new angiographic lesions, ischemic events, and changes in therapy for TAK. At baseline assessment, 36 TAK patients underwent 18F-FDG PET-CT scan and maximal standardized uptake value (SUVmax) in arteries ≥ 1.3 was predictive for clinical disease activity. Thirty-two TAK patients were then followed-up for a median 83.5 months. Twenty-three (71.9%) patients developed clinical relapses and new arterial lesions were observed in 14 (43.8%) cases. Disease relapses [85.0% vs. 50.0%, p = 0.049; odds ratio (OR): 5.667; 95% confidence interval (95 CI): 1.067-30.085] and the need for changing immunosuppressive therapy (85.0% vs. 41.7%, p = 0.018; OR: 7.933; 95CI: 1.478-42.581) were more frequently found in patients with SUVmax ≥ 1.3 at baseline compared with those presenting SUVmax < 1.3. No associations were found between SUVmax ≥ or < 1.3 in large arteries at baseline and the development of ischemic events, sustained remission or new angiographic lesions. In multivariate analysis, associations between baseline SUVmax ≥ 1.3 and disease relapses were not independent (hazard ratio: 1.07; 95 CI 0.39-2.92; p = 0.892). In conclusion, arterial SUVmax is marginally associated with disease relapses and with the need to change therapy in TAK. 18F-FDG uptake in large arteries is not associated with the development of new arterial lesions in TAK.

Clinical characteristics and long-term outcome of Takayasu arteritis in Iran: a multicentre study

Background/aim: This study aimed to evaluate the demographic, clinical, angiographic and prognostic characteristics of Takayasu arteritis (TA) in Iran. Materials and methods: A total of 75 patients with TA based on the American College of Rheumatology 1990 criteria for TA classification referred to the Rheumatology Centres, were followed-up from 1989 to 2019. Demographic, clinical, angiographic and prognostic characteristics were collected at baseline and last visit. Results: The mean age was 31.9 ± 9.8 years at the disease onset. Female to male ratio was 14. The median latency in diagnosis was 24 months. Pulse discrepancy in the arms, blood pressure discrepancy in the arms, limb claudication, hypertension and constitutional symptoms were the most common clinical features. The most common angiographic type at the time of diagnosis was Type I (42.7%). The most frequent arterial lesion was stenosis (89.4%). Subclavian, carotid and aortic arteries were the most commonly involved arteries. New lesions developed in 28.6% of patients during the 5.25-year follow-up. Vasculitis-induced chronic damage was observed in all patients. Disease activity decreased and vascular damage remained stable throughout the follow-up period. Conclusions: The clinical features and angiographic type of TA in Iran are different from most Asian countries. Differences in angiographic and clinical features may lead to delayed diagnosis. The issue of delay in diagnosis should create awareness among health care providers that TA is not a very rare disease in Iranians and failure to pay attention to warning symptoms may delay the diagnosis.

Effectiveness of Tocilizumab in juvenile patients with refractory Takayasu arteritis: Two case reports.

RATIONALE: Takayasu arteritis (TA) is a systemic large-vessel vasculitis which can be accompanied by the symptoms associated with vascular stenosis. PATIENT CONCERNS: We describe 2 female juveniles with TA who presented with progressive intermittent claudication. DIAGNOSIS: Contrast-enhanced computed tomography (CT) revealed the stenosis of femoral arteries and increased levels of C-reactive protein (CRP), and serum amyloid A (SAA) were noted in both patients. According to European league against rheumatism consensus criteria for the diagnosis of TA was confirmed in both patients. INTERVENTIONS: Both patients had shown resistance to glucocorticoids and treated with tocilizumab (TCZ) (subcutaneous injections, 162 mg/week). OUTCOMES: These treatments improved claudication symptoms. Follow-up imaging by enhanced CT revealed restoration of advanced stenosis of the femoral arteries in both patients. They achieved normalization of levels of the acute-phase reactants CRP and SAA. Serum levels of interleukin-6 were increased transiently after TCZ injection, but declined to within normal ranges at 12 weeks. LESSONS: Juvenile patients with TA presenting with advanced stenosis of the femoral arteries are not rare. The clinical courses of our patients suggested the beneficial effects of TCZ against the progressive vascular stenosis observed in refractory TA.

Vascular ultrasound for monitoring of inflammatory activity in Takayasu arteritis.

BACKGROUND: Takayasu arteritis (TA) is a rare large-vessel arteritis that primarily affects the aorta and its major branches. The aim of this study was to describe the value of high frequency ultrasound for monitoring of inflammatory activity. METHODS: Twenty-five patients, range 11-71 years, diagnosed with TA were investigated with duplex ultrasound (DUS) including follow-up studies. Twenty-five healthy controls were also investigated. Nine patients had newly diagnosed active TA. Sixteen patients had stable/inactive disease at baseline DUS, and TA was diagnosed median 4·5 years previously. Intima-media thickness (IMT), vessel and lumen diameter were measured in the carotid arteries, central neck arteries and the aortic arch. The vessel walls were studied qualitatively. The Takayasu ultrasound index was created for inflammatory activity scoring. RESULTS: Intima-media thickness in common carotid artery (CCA) was (median and 25-75 percentile parenthetic) 2·3 mm (1·7-2·9) in clinically active TA, 1·2 mm (1·1-1·6) in clinically stable TA (P<0·001) and 0·5 mm (0·5-0·6) in healthy controls (P<0·001). Clinically active TA had prominent increase in IMT and/or increased vessel diameter, and/or intramural arteries, and/or hypoechogenic areas interpreted as oedema in the vessel wall. TA in clinical remission was characterized by increased IMT with medium to high echogenicity with or without fibrotic stripes. The Takayasu ultrasound index was higher in patients with active disease versus treated disease, 2·55 (1·60-3·05) versus 1·30 (1·00-1·58), (P = 0·003). CONCLUSION: DUS is an excellent tool to monitor inflammatory changes in the vessel wall in TA. Further DUS studies in larger patient populations are warranted.

Typical angina in a patient with Takayasu arteritis.

Takayasu arteritis (TA) is a well-reported form of large-vessel vasculitis that primarily affects the aorta and its major branches. Cardiac manifestations of TA have been reported and can include typical angina secondary to coronary artery involvement; however, typical angina in the setting normal coronary arteries is uncommon. We describe a case of typical angina in a patient with TA with normal coronary arteries secondary to diastolic hypotension, in the absence of significant aortic regurgitation, likely from poor aortic distensibility and elevated left ventricular end-diastolic pressure.

Midaortic Syndrome due to Takayasu Arteritis in a Child with Acute Decompensated Cardiac Failure Managed by an Emergency Axillo-External Iliac Artery Bypass: A Follow-Up Case Report of Long-Term Outcomes.

Midaortic syndrome (MAS) is characterized by a diffuse narrowing of the distal thoracic or abdominal aorta and is concomitant with various etiologies. The common symptoms of MAS include severe hypertension or arterial insufficiency distal to the stenosis. This includes lower extremity claudication and heart failure due to afterload mismatch. We present the case of an 8-year-old girl who developed acute decompensated cardiac, respiratory, and renal failures because of the occlusion of the descending aorta secondary to Takayasu arteritis (TA). Although thoracoabdominal-aortic bypass is usually performed for patients with MAS, the procedure was considered too invasive, given the patient's condition. Therefore, we performed an emergency axillo-external iliac artery bypass for revascularization. Subsequently, organ failure improved and she was discharged. At postoperative 10 years, an asymptomatic pseudoaneurysm was detected at the distal anastomosis, for which revision surgery was performed. Overall, the long-term prognosis was satisfactory, suggesting that this procedure is less invasive and effective for treatment of MAS due to TA, in emergencies.