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Background and purpose: Systemic lupus erythematosus (SLE) is associated with significant cerebrovascular and neuropsychiatric disease for which multiple pathogeneses have been proposed. Although global cerebral hypoperfusion has been proposed, there are limited data about intracerebral arterial hemodynamics. Transcranial Doppler (TCD) allows portable, high temporal and spatial resolution, noninvasive blood velocity measurements in the middle cerebral arteries, and calculations of standard resistivity (RI) and pulsatility (PI) indices. RI and PI correlate with cerebral hemispheric arteriolar tone, blood flow resistances, and impedances. Accordingly, we hypothesized that there would be significant differences (p < 0.05) in RI and PI between SLE patients and healthy, age and gender matched controls. Methods: TCD was used to measure RI and PI bilaterally on 34 stable SLE patients (35 ± 11 years) and 15 control subjects (34 ± 10 years). Patients and controls had similar, normal blood pressures and were examined in the supine position during normal, resting respiration. RI and PI were determined by a blinded, experienced observer. Results: There were no significant differences in RI and PI bilaterally within each cohort. However, SLE patients had significantly lower average RI and PI values compared with controls: 0.45 ± 0.10 versus 0.52 ± 0.05 (p < 0.05); and 0.65 ± 0.19 versus 0.77 ± 0.12, (p < 0.05); respectively. Conclusions: These preliminary data suggest that RI and PI values in the human middle cerebral artery are significantly lower in SLE compared with controls. These indices indicate that middle cerebral arterial resistances and impedances are decreased in SLE. Under normotensive conditions, the results are consistent with hyperperfusion in SLE with increased arteriolar dilation and increased cerebral blood flow. [ABSTRACT FROM AUTHOR]

BackgroundAedes aegypti is a primary vector of dengue, chikungunya, Zika, and urban yellow fever viruses. Indoor, ultra low volume (ULV) space spraying with pyrethroid insecticides is the main approach used for Ae. aegypti emergency control in many countries. Given the widespread use of this method, the lack of large-scale experiments or detailed evaluations of municipal spray programs is problematic.Methodology/principal findingsTwo experimental evaluations of non-residual, indoor ULV pyrethroid spraying were conducted in Iquitos, Peru. In each, a central sprayed sector was surrounded by an unsprayed buffer sector. In 2013, spray and buffer sectors included 398 and 765 houses, respectively. Spraying reduced the mean number of adults captured per house by ~83 percent relative to the pre-spray baseline survey. In the 2014 experiment, sprayed and buffer sectors included 1,117 and 1,049 houses, respectively. Here, the sprayed sector's number of adults per house was reduced ~64 percent relative to baseline. Parity surveys in the sprayed sector during the 2014 spray period indicated an increase in the proportion of very young females. We also evaluated impacts of a 2014 citywide spray program by the local Ministry of Health, which reduced adult populations by ~60 percent. In all cases, adult densities returned to near-baseline levels within one month.Conclusions/significanceOur results demonstrate that densities of adult Ae. aegypti can be reduced by experimental and municipal spraying programs. The finding that adult densities return to approximately pre-spray densities in less than a month is similar to results from previous, smaller scale experiments. Our results demonstrate that ULV spraying is best viewed as having a short-term entomological effect. The epidemiological impact of ULV spraying will need evaluation in future trials that measure capacity of insecticide spraying to reduce human infection or disease.

Este artículo presenta unos lineamientos de gestión importantes para llevar a cabo un proceso de planeación turística en el corregimiento de San Francisco de Asís, los cuales fueron desarrollados a partir de la noción conceptual de turismo sostenible y la participación de los pobladores locales. Para este caso, se consideró el método de la investigación participativa, se utilizaron técnicas de la etnografía y se implementaron talleres con la comunidad. Como resultado principal se propone la consolidación de cuatro componentes, el socio- político, el socio- cultural, el socio- ecológico y el socio- económico, que deberán gestionarse de manera integral para alcanzar una verdadera sostenibilidad turística en este territorio. En el primer componente, se esboza un proceso participativo que deberá llevarse a cabo entre los diferentes actores- sectores involucrados; en el segundo, se plantea fortalecer el tejido social y valorizar las tradiciones culturales para integrarlas adecuadamente con las actividades turísticas; en el tercero, se propone una conservación ambiental integral en todo el territorio y como una obligación de todos los actores- sectores involucrados, incluyendo también a los turistas; por último, en el cuarto componente, se recomienda la generación de más oportunidades y de bienestar general para toda la población local, con el fin de evitar la proliferación de un turismo segregado. Como conclusión general, se proyecta que la implementación de estos lineamientos permitirá avanzar, desde lo práctico, en el desarrollo de un auténtico turismo sostenible que ayudará a mejorar las condiciones de vida de la población local y a conservar los valiosos recursos naturales y culturales que existen en este territorio.

Abstract Background Neuropsychiatric systemic lupus erythematosus (NPSLE) is associated with increased morbidity and mortality. Methods We used Diffusion Tensor Imaging (DTI) to assess white matter abnormalities in seventeen NPSLE patients, sixteen SLE patients without NPSLE, and twenty age- and gender-matched controls. Results NPSLE patients differed significantly from SLE and control patients in white matter integrity of the body of the corpus callosum, the left arm of the forceps major and the left anterior corona radiata. Conclusions Several possible mechanisms of white matter injury are explored, including vascular injury, medication effects, and platelet or fibrin macro- or microembolism from Libman-Sacks endocarditis.

Preoperative management of a pheochromocytoma involves α-adrenergic receptor blockers and volume expansion followed by β-adrenergic receptor blockers and then adrenalectomy, which in the presence of very severe aortic valve stenosis would be challenging. We describe a patient with this rare combination who underwent pharmacotherapy followed by transcatheter aortic valve replacement and then adrenalectomy, culminating in a successful outcome for this patient., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Introduction: There is limited human imaging data on the association of adventitial thickness (AT) with arterial disease. Systemic lupus erythematosus (SLE) is a prototypical disease model for studying markers of premature arterial disease., Objective: To determine if increased aortic AT is associated with aortic atherosclerosis [increased intima media thickness (IMT) or plaques], stiffness [increased pressure-strain elastic modulus (PSEM)], and vessel remodeling., Methods: In total, 70 SLE patients and 26 age- and sex-matched controls underwent transesophageal echocardiography (TEE). Two-dimensional guided M-mode images were obtained to assess AT, IMT, and plaques, and PSEM at the proximal, mid, and distal thoracic aorta. Images were interpreted by 3 observers unaware of the subjects' clinical data and each other's measurements. Abnormal aortic AT, IMT, and PSEM were defined as > 2SD above the overall mean values in controls and corresponded to > 1 mm, > 1 mm, and > 10.6 Pascal units, respectively. Plaques were defined as focal-protruding IMT > 50% of the surrounding vessel wall., Results: Abnormal aortic AT, atherosclerosis, and abnormal stiffness were more frequent in SLE patients than in controls (all p ≤ 0.02). In SLE patients, abnormal AT combined with atherosclerosis was associated with larger aortic end-diastolic diameters than in controls (p ≤ 0.05). In SLE patients, aortic AT was greater in patients with atherosclerosis and in those with abnormal stiffness than in patients without these abnormalities (all p ≤ 0.02). In patients with abnormal AT, the degree of aortic stiffness was similar to those with atherosclerosis (p = 0.22)., Conclusion: In patients with SLE, increased aortic AT is associated with aortic atherosclerosis, abnormal stiffness, and eccentric vessel remodeling. Key Points • In patients with SLE, abnormal aortic adventitial thickness is associated with aortic atherosclerosis, abnormal stiffness, and eccentric vessel remodeling. • In patients with SLE, aortic adventitial thickening may contribute to the extent of aortic atherosclerosis, abnormal aortic stiffness, and vessel remodeling. • To our knowledge, this is the first human imaging study to characterize the aortic adventitial layer and delineate its association with aortic disease.

Background: Libman-Sacks endocarditis in patients with systemic lupus erythematosus (SLE) is commonly complicated with embolic cerebrovascular disease (CVD) or valve dysfunction for which high-risk valve surgery is frequently performed. However, the role of medical therapy alone for Libman-Sacks endocarditis and associated acute CVD remains undefined., Objective: To determine in this cross-sectional and longitudinal study if conventional anti-inflammatory and anti-thrombotic therapy may be an effective therapy in SLE patients with Libman-Sacks endocarditis and associated acute CVD., Methods and Materials: 17 SLE patients with Libman-Sacks endocarditis detected by two-and-three-dimensional transesophageal echocardiography (TEE) and complicated with acute CVD [stroke/TIA, focal brain injury on MRI, or cognitive dysfunction] were treated with conventional anti-inflammatory and anti-thrombotic therapy for a median of 6 months and then underwent repeat TEE, transcranial Doppler, brain MRI, and neurocognitive testing for re-assessment of Libman-Sacks endocarditis and CVD., Results: Valve vegetations decreased in number, diameter, and area (all p ≤0.01); associated valve regurgitation significantly improved (p = 0.04), and valve thickening did not progress (p = 0.56). In 13 (76%) patients, valve vegetations or valve regurgitation resolved or improved in number and size or by ≥1 degree, respectively, as compared to 4 (24%) patients in whom vegetations or valve regurgitation persisted unchanged or increased in size or by ≥1 degree (p = 0.03). Also, cerebromicroembolism, lobar and global gray and white matter cerebral perfusion, ischemic brain lesion load, and neurocognitive dysfunction resolved or significantly improved (all p ≤0.04)., Conclusion: These preliminary data suggest that combined conventional anti-inflammatory and antithrombotic therapy may be an effective treatment for Libman-Sacks endocarditis and its associated CVD and may obviate the need for high-risk valve surgery., Competing Interests: The authors have declared that no competing interests exist.

A 66-year-old gentleman with no prior cardiac history presented with dyspnea on exertion and chest pain for 1 month. His workup included a transthoracic echocardiogram, which demonstrated findings suggestive of cor triatriatum (C-TAT) with uncertain degree of hemodynamic obstruction. In addition, mild left ventricular systolic dysfunction and segmental wall motion abnormalities suggestive of coronary artery disease were noted. The patient then underwent transesophageal echocardiography (TEE) to define better the structural characteristics and hemodynamic significance of the C-TAT, left and right heart catheterization to assess pressure gradients between the upper and lower left atrial chambers by simultaneous measurement of pulmonary capillary wedge and left ventricular end-diastolic pressures, and coronary angiography. Multiplane 2-dimensional TEE demonstrated an atrial membrane extending from the inferior portion of the interatrial septum to the superior aspect of the lateral atrial wall. This membrane exhibited a medial large oval opening with bidirectional flow and a ≤2 mm Hg gradient. Three-dimensional TEE imaging re-demonstrated this obliquely oriented membrane; however, of most importance, it revealed that the membrane divided the atria into a medial funnel and C-shaped cavity with a large distal oval shape opening and an even larger lateral atrial cavity. These findings were inconsistent with a true C-TAT and rather demonstrated what we defined as a pseudo-C-TAT membrane. Simultaneous right and left heart catheterization confirmed a minimal gradient of 3 to 5 mm Hg and coronary angiography demonstrated severe 3-vessel coronary disease as the primary cause of the patient's clinical syndrome.

Background: Transesophageal echocardiography (TEE) has not been compared to transthoracic echocardiography (TTE) for assessment of left ventricular diastolic function (LVDF). Left ventricular diastolic dysfunction is common in systemic lupus erythematosus (SLE), a disease model of premature myocardial disease., Methods: 66 patients with SLE (mean age 36±12 years, 91% women) and 26 age-and-sex matched healthy volunteers (mean age 34±11 years, 85% women) underwent TEE immediately followed by TTE. From basal four-chamber views, mitral inflow E and A velocities, E/A ratio, E deceleration time, isovolumic relaxation time, septal and lateral mitral E' and A' velocities, septal E'/A' ratio, mitral E to septal and lateral E' ratios, and pulmonary veins systolic to diastolic peak velocities ratio were measured. Measurements were averaged over 3 cardiac cycles and performed by 2 independent observers., Results: LVDF parameters were worse in patients than in controls by TEE and TTE (all p≤0.03). Most LVDF parameters were similar within each group by TEE and TTE (all p≥0.17). By both techniques, mitral E and A, mitral and septal E/A ratios, septal and lateral E', septal and lateral E/E' ratios, and average E/E' ratio were highly correlated (r=0.64-0.96, all p≤0.003); E deceleration time, isovolumic relaxation time, and septal A' velocities were moderately correlated (r=0.43-0.54, all p≤0.03); and pulmonary veins systolic to diastolic ratio showed the lowest correlation (r=0.27, p=0.04)., Conclusion: By TEE and TTE, LVDF parameters were worse in SLE patients than in controls; and in both groups, LVDF parameters assessed by TEE and TTE were similar and significantly correlated., Competing Interests: Conflicts of Interest None.

The progression of atherosclerosis versus arterial stiffness with age within and between arteries has not been defined. Systemic lupus erythematosus (SLE) is a human model of accelerated arterial disease that may permit this determination. 76 SLE patients (69 women, age 37 ± 12 years) and 26 age-and-sex-matched controls (22 women, age 34 ± 11 years) underwent transesophageal echocardiography and carotid ultrasonography for assessment of atherosclerosis [plaques and intima-media thickening (IMT)] and arterial stiffness [increased pressure-strain elastic modulus (PSEM)] of the descending thoracic aorta and carotid arteries. Since IMT is highly associated with plaques, IMT was used as a marker of atherosclerosis to assess its progression in relation with age and PSEM. Aortic and carotid plaques, IMT, and PSEM were greater in patients than in controls (all p ≤ 0.05). Within the aorta and within the carotid arteries, the average percent increases per decade of age for IMT versus PSEM were similar in patients (8.55% versus 9.33% and 3.39% versus 2.46%, respectively) and controls (5.53% versus 6.60% and 4.75% versus 3.49%, respectively) (all p ≥ 0.58). However, in SLE patients, the average percent increases per decade of age for IMT and PSEM were higher in the aorta than in the carotid arteries (8.55% and 9.33% versus 3.39% and 2.46%, respectively, both p ≤ 0.03). In patients with SLE, atherosclerosis and arterial stiffness progress with age parallel to each other within arteries, but divergently between arteries with different anatomy and hemodynamics.

Neurocognitive dysfunction and brain injury on magnetic resonance imaging (MRI) are common in patients with systemic lupus erythematosus (SLE) and are associated with increased morbidity and mortality. However, brain MRI is expensive, is restricted by payers, and requires high expertise. Neurocognitive assessment is an easily available, safe, and inexpensive clinical tool that may select patients needing brain MRI. In this cross-sectional and controlled study, 76 SLE patients (69 women, age 37 ± 12 years) and 26 age and gender-matched healthy subjects (22 women, age 34 ± 11 years) underwent assessment of attention, memory, processing speed, executive function, motor function, and global neurocognitive function. All subjects underwent brain MRI with T1-weighted, fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging. Hemispheric and whole brain lesion load in cm 3 were determined using semi-automated methods. Neurocognitive z-scores in all clinical domains were significantly lower and whole brain and right and left hemispheres brain lesion load were significantly greater in patients than in controls (all p ≤ 0.02). There was significant correlation between neurocognitive z-scores in all domains and whole brain lesion load: processing speed (r = - 0.46; p < 0.0001), attention (r = - 0.42; p < 0.001), memory (r = - 0.40; p = 0.0004), executive function (r = - 0.25; p = 0.03), motor function (r = - 0.25; p = 0.05), and global neurocognitive function (r = - 0.38; p = 0.006). Similar correlations were found for brain hemisphere lesion loads (all p ≤ 0.05). These correlations were strengthened when adjusted for glucocorticoid therapy and SLE disease activity index. Finally, global neurocognitive z-score and erythrosedimentation rate were the only independent predictors of whole brain lesion load (both p ≤ 0.007). Neurocognitive measures and brain lesion load are worse in SLE patients than in controls. In SLE patients, neurocognitive z-scores correlate negatively with and independently predict brain lesion load. Therefore, neurocognitive testing may be an effective clinical tool to select patients needing brain MRI.

A previously asymptomatic young female with no previous medical or cardiac history collapsed during indoor exercise. A portable automatic external defibrillator showed a shockable rhythm. She received multiple electrical shocks with return to normal sinus rhythm without ischaemic ECG changes. Her troponin level was mildly elevated. A transthoracic echocardiogram revealed moderately reduced left ventricular ejection fraction with global hypokinesis. During emergent coronary angiography, the left main coronary artery could not be found. The right coronary artery was normal with robust collaterals to the entire left coronary circulation extending to the left main coronary artery, but did not fill the ostium. Coronary CT angiogram confirmed nearly complete absence of the left main coronary artery ostium. A diagnosis of left main coronary artery atresia was made. Patient underwent successful two vessel coronary artery bypass grafting. She continues to do well 1 year postoperatively., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

The benefit of intracoronary thrombolytics in ST-elevation myocardial infarction (STEMI) is not well established. Mainstays of STEMI management include intravenous thrombolytics, percutaneous coronary interventions and surgical revascularisation. However, in cases of STEMI secondary to coronary artery aneurysmal disease (CAAD), standard treatment options may not be suitable due to high thrombus burden, perioperative risk and factors unique to each patient. Thus, STEMI management in CAAD can represent a therapeutically challenging clinical scenario. Here, we describe a patient with severe three-vessel CAAD complicated by multivessel thrombosis and cardiogenic shock for whom traditional management options including placement of haemodynamic support devices were not feasible. As an alternative measure, the patient was treated with intracoronary thrombolysis with remarkable clinical stabilisation and angiographic resolution of thrombosis. He remains clinically stable several years later without recurrent events. This case serves to demonstrate the potential lifesaving benefit of intracoronary thrombolysis in complicated multivessel CAAD., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

This article presents the case of a 53-year-old man who presented with acute right superficial femoral and popliteal arterial thrombosis for which he underwent an emergent uncomplicated thrombectomy. He denied preceding cardiovascular or neurologic symptomatology and had no history of coronary or peripheral arterial disease, trauma, hypercoagulability, or malignancy. However, he reported having several days of intense emotional stress prior to presentation. His cardiac exam was normal, his electrocardiogram showed normal sinus rhythm and nonspecific ST-T wave abnormalities, and his troponin levels were normal. Transthoracic echocardiography (TTE) revealed a large (2.4 × 2 cm) apical left ventricle (LV) thrombus, LV apical akinesis, and LV ejection fraction of 40% to 45%. Coronary angiography revealed only luminal irregularities. A repeat TTE performed 3 days after initiating unfractionated heparin revealed complete resolution of the LV thrombus. The patient had an uneventful clinical course and was discharged home in stable condition on oral anticoagulants. The lower incidence of LV thrombus in takotsubo cardiomyopathy (TC) of 1.3% in comparison to 4% to 8% in acute myocardial infarction due to coronary artery disease in the current era of early reperfusion may be explained by the lower extent of ischemic myocardial necrosis associated with TC. This case suggests that the lower extent of myocardial necrosis in TC may also lead to faster resolution of LV thrombus. Therefore, earlier follow-up with TTE (within 2 weeks) and shorter duration of anticoagulation (<3 months) may be considered in patients with TC complicated by LV thrombus formation with or without systemic embolism., Competing Interests: Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

We report a case of hypermagnesemia associated with the use of milk of magnesia in a male patient with end-stage renal disease. After experiencing nausea and vomiting, he developed severe bradycardia and then asystole. Resuscitation efforts were successful; however, he developed atrial fibrillation with severe widening of the QRS and diffuse ST elevation, hypothermia, hypotension and apnoea requiring intubation. Initial diagnoses included ST-elevation myocardial infarction, cardiogenic and/or septic shock and hyperkalaemia. However, serum magnesium was later found to be >4.1 mmol/L (equivalent to >10 mg/dL). He underwent haemodialysis (HD) to remove serum magnesium with remarkable overall improvement. Severe hypermagnesemia can manifest with severe bradycardia and asystole, shock, hypothermia and respiratory failure and can mimic acute coronary syndromes complicated with cardiogenic shock or septic shock. Therefore, clinicians should be aware of this life-threatening condition in patients with significant renal dysfunction. Timely treatment with HD is highly effective and lifesaving., (2017 BMJ Publishing Group Ltd.)

Pancreaticopericardial fistula (PPF) is a rare subset of thoracopancreatic fistulas with few reported cases in the literature. Historically, treatment of PPF has included pancreatic ductal stenting or surgery, or both, but we present a case of PPF that was successfully treated using only pericardial and pancreatic drains., (Published by Elsevier Inc.)

The patient is a 70-year-old male with no other atherogenic risk factors who presented with an acute coronary syndrome (ACS) of unstable angina subsequently complicated by a non-ST elevation myocardial infarction (NSTEMI). The patient's presentation posed 3 unique features: (1) cardiac catheterization demonstrated nonobstructive 3-vessel multi-aneurysmal coronary artery disease with sluggish antegrade coronary flow; (2) a nonobstructive aneurysmal dissection flap based on contrast staining of the mid left anterior descending artery, which may have led to in situ nonocclusive thrombosis and distal microvascular embolization; and (3) successful conservative medical therapy of coronary artery aneurysmal disease (CAAD) complicated with ACS. CAAD has an incidence of 1.5% to 4.9% in adults. The most common etiology of CAAD is atherosclerotic coronary artery disease. There are no guidelines for the management of CAAD complicated by ACS, and controversies exist as to whether conservative, catheter-based, or surgical management should be pursued.

Aortic valve infective endocarditis (IE) can be complicated with severe aortic regurgitation (AR) jet-related lesions such as vegetations, pseudoaneurysms, aneurysms or perforations on the anterior mitral leaflet. Herein is reported the case of a 69-year-old male with culture-negative aortic valve endocarditis complicated with aortic valve perforations resulting in severe and eccentric AR and an AR jet-related ventricular septal defect (VSD). Neither transthoracic echocardiography (TTE) nor two-dimensional transesophageal echocardiography (2D-TEE) were unable to clearly discriminate an aorto-right ventricular fistula from a VSD. By contrast, three-dimensional TEE (3D-TEE) demonstrated multiple aortic valve vegetations, aortic valve perforations, severe AR, and an AR jet-related VSD. The 3D-TEE findings were confirmed at surgery, the aortic valve was replaced with a bioprosthetic valve, and the VSD was repaired with a pericardial patch. To the authors' knowledge, this is the first case of a VSD resulting from an AR jet lesion to be detected and characterized using 3D-TEE. The accurate preoperative diagnosis and characterization of the VSD with 3D-TEE contributed to successful surgery and the patient's short- and long-term survival. Video 1A: This four-chamber transthoracic echocardiography (TTE) view with color Doppler shows a color-Doppler jet traversing the basal interventricular septum (IVS) into the right ventricle, predominantly during systole. However, the origin of the jet resulting from an aortic-right ventricular fistula cannot be determined. A mild to moderate degree of tricuspid regurgitation is also noted. Video 1B: Close-up view of the basal IVS demonstrates a color Doppler jet traversing the IVS, predominantly during systole and apparently originating in the left ventricular outflow tract side, as illustrated by a color Doppler acceleration zone. However, and as in Fig. 1A, the origin of the jet resulting from an aortic-right ventricular fistula cannot be determined. Video 1C: Color Doppler two-dimensional TEE view longitudinal to the aortic valve and aortic root, showing a predominantly systolic color Doppler jet originating in the left ventricular outflow tract and/or aorta and crossing the anterior and basal IVS defect into the right ventricle. Videos 1D: These three-dimensional TEE en-face left ventricular outflow tract views of the aortic valve and basal anterior IVS (Video 1D) and medial anterior close up (Video 1E) and lateral en-face (Video 1F) views of the LV outflow tract and IVS clearly define the anterior and basal location of the VSD with irregular borders and a residual mobile tissue fragment. Multiple aortic valve vegetations and a perforation are visible in Video 1D. Videos 1E: These three-dimensional TEE en-face left ventricular outflow tract views of the aortic valve and basal anterior IVS (Video 1D) and medial anterior close up (Video 1E) and lateral en-face (Video 1F) views of the LV outflow tract and IVS clearly define the anterior and basal location of the VSD with irregular borders and a residual mobile tissue fragment. Multiple aortic valve vegetations and a perforation are visible in Video 1D. Videos 1F: These three-dimensional TEE en-face left ventricular outflow tract views of the aortic valve and basal anterior IVS (Video 1D) and medial anterior close up (Video 1E) and lateral en-face (Video 1F) views of the LV outflow tract and IVS clearly define the anterior and basal location of the VSD with irregular borders and a residual mobile tissue fragment. Multiple aortic valve vegetations and a perforation are visible in Video 1D.

Background: Libman-Sacks endocarditis, characterized by Libman-Sacks vegetations, is common in patients with systemic lupus erythematosus and is commonly complicated with embolic cerebrovascular disease. Thus, accurate detection of Libman-Sacks vegetations may lead to early therapy and prevention of their associated complications. Although two-dimensional (2D) transesophageal echocardiography (TEE) has high diagnostic value for detection of Libman-Sacks vegetations, three-dimensional (3D) TEE may allow improved detection, characterization, and clinical correlations of Libman-Sacks vegetations., Methods: Twenty-nine patients with systemic lupus erythematosus (27 women; mean age, 34 ± 12 years) prospectively underwent 40 paired 3D and 2D transesophageal echocardiographic studies and assessment of cerebrovascular disease manifested as acute clinical neurologic syndromes, neurocognitive dysfunction, or focal brain injury on magnetic resonance imaging. Initial and repeat studies in patients were intermixed in a blinded manner with paired studies from healthy controls, deidentified, coded, and independently interpreted by experienced observers unaware of patients' clinical and imaging data., Results: The results of 3D TEE compared with 2D TEE were more often positive for mitral or aortic valve vegetations, and 3D TEE detected more vegetations per study and determined larger sizes of vegetations (P ≤ .03 for all). Also, 3D TEE detected more vegetations on the anterior mitral leaflet, anterolateral and posteromedial scallops, and ventricular side or both atrial and ventricular sides of the leaflets (P < .05 for all). In addition, 3D TEE detected more vegetations on the aortic valve left and noncoronary cusps, coronary cusps' tips and margins, and aortic side or both aortic and ventricular sides of the cusps (P ≤ .01 for all). Furthermore, 3D TEE more often detected associated mitral or aortic valve commissural fusion (P = .002). Finally, 3D TEE detected more vegetations in patients with cerebrovascular disease (P = .01)., Conclusions: Three-dimensional TEE provides clinically relevant additive information that complements 2D TEE for the detection, characterization, and association with cerebrovascular disease of Libman-Sacks endocarditis., (Published by Elsevier Inc.)

Background: Lambl's excrescences (LEx) are detected by transesophageal echocardiography (TEE) and are characterized as thin, elongated, and hypermobile structures located at the leaflets' coaptation point of the heart valves. The association of LEx with cerebrovascular disease (CVD) is still undefined and yet patients with LEx and suspected CVD receive unproven effective antiplatelet or anticoagulant therapy or even undergo valve surgery. Also, the association of LEx with aging and atherogenic, inflammatory, or thrombogenic parameters has not been reported., Methods: Seventy-seven patients with systemic lupus erythematosus (SLE) (71 women, age 37 ± 12 years) and 26 age- and sex-matched healthy controls (22 women, age 34 ± 11 years) prospectively underwent routine history and physical exam, transcranial Doppler, brain MRI, TEE, carotid duplex, and clinical and laboratory evaluations of atherogenesis, inflammation, platelet activity, coagulation, and fibrinolysis. Subjects without stroke/TIA on enrollment (with and without LEx) had a median follow-up of 57 months., Results: On enrollment, 33 (43%) of 77 patients had CVD manifested as acute stroke/TIA (23 patients), cerebromicroembolism by transcranial Doppler (17 patients), or cerebral infarcts by MRI (14 patients). Mitral or aortic valve LEx were equally frequent in healthy controls (46%) as in patients with and without any CVD (39 and 43%), stroke/TIA (35 and 43%), cerebromicroembolism (41 and 42%), or cerebral infarcts (36 and 43%) (all p ≥ 0.72). Also, other mechanisms for CVD other than LEx such as Libman-Sacks vegetations, patent foramen ovale or interatrial septal aneurysm, aortic or carotid atherosclerosis, or thrombogenesis were found in ≥94% of patients with CVD. In addition, 36 subjects with and 44 without LEx had similar low incidence of stroke/TIA (1 (1.3%) and 2 (2.5%), respectively, p = 1.0) during follow-up. Finally, LEx were not associated with aging, atherogenic risk factors, atherosclerosis, inflammation, or thrombogenesis., Conclusions: In this study, LEx are similarly prevalent in healthy controls and SLE patients, are not associated with CVD, and are not associated with pathogenic risk factors. Therefore, the study findings suggest that LEx may not be cardioembolic substrates, may not represent pathologic valve structures, and may not require therapy.

Non-invasive transcranial Doppler (TCD) is widely used for blood velocity (BV, cm/sec) measurements in the human middle cerebral artery (MCA). MCABV measurements are accepted as linear with MCA blood flow (MCABF). Magnetic resonance angiography (MRA) provides measurements of MCA lumen diameters that can be combined with TCD MCABV to calculate MCABF (mL/min). We tested the precision and accuracy of this method against a flow phantom and in vivo proximal internal carotid artery blood flow (ICABF). In vitro precision (repeated measures) and accuracy (vs. time collection) gave correlations coefficients of 0.97 and 0.98, respectively (both p < 0.05). In vivo precision (repeated measures) and accuracy (vs. ICABF) gave correlation coefficients of 0.90 (left and right), 0.94 (left) and 0.93 (right) (all p < 0.05). Bilateral MCABF in 35 adults were similar (left, 168 ± 72 mL/min; right, 180 ± 69 mL/min; p > 0.05). Results suggest that blood velocity by TCD and lumen diameter by MRA can be combined to estimate absolute values of MCABF., (Copyright © 2014 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.)

A 51-year-old male with history of resected renal cell carcinoma and prior pulmonary embolism presented with tachypnea, tachycardia and progressive dyspnea on exertion. Chest computed tomography revealed bilateral acute pulmonary embolism. Transthoracic echocardiogram showed severe pulmonary hypertension with severe cor-pulmonale and presence of a large worm-like thrombus extending across the foramen ovale, entering both ventricles through the mitral and tricuspid valves. The risks of anti-coagulation, pharmacologic thrombolysis, and surgical thrombectomy, in a hemodynamically stable patient, posed a significant therapeutic dilemma. Ultimately, a collective decision was made to start anticoagulation, without incident. At 1 month follow up, complete resolution of the intracardiac thrombus, pulmonary hypertension, and cor-pulmonale were observed with full clinical recovery of the patient.

Background: Aortic stiffness and left ventricular (LV) diastolic dysfunction are common and associated with increased morbidity and mortality in systemic lupus erythematosus (SLE)., Hypothesis: In SLE, aortic stiffness and LV diastolic dysfunction may be associated., Methods: This 6-year-duration, cross-sectional, and controlled study was conducted in 76 SLE patients (69 women; mean age, 37 ± 12 years) and 26 age- and sex-matched healthy controls. All subjects underwent clinical and laboratory evaluations and transesophageal echocardiography (TEE) to assess LV diastolic function and stiffness of the descending thoracic aorta using the pressure-strain elastic modulus (PSEM). To validate results using PSEM, aortic strain, stiffness, and distensibility were assessed., Results: Patients as compared with controls had higher PSEM (8.14 ± 4.25 vs 5.97 ± 2.31 U, P < 0.001) and had lower mitral inflow E/A and septal and lateral mitral annulus tissue Doppler E'/A' velocity ratios, longer isovolumic relaxation time, lower septal and lateral mitral annulus E' velocities, and higher mitral E/septal E' and mitral E/lateral E' velocity ratios (all P ≤ 0.03), all indicative of LV diastolic dysfunction. In patients, PSEM was correlated with parameters of LV diastolic dysfunction (all P < 0.05), was independently negatively associated with E/A and E'/A' ratios and E' velocities, and was positively associated with E/E' ratios (P ≤ 0.02 for each parameter and P < 0.001 for all parameters as a profile). Aortic strain, stiffness, and distensibility were also worse in patients than in controls (all P < 0.05) and were correlated with parameters of LV diastolic dysfunction (all P ≤ 0.03)., Conclusions: Aortic stiffness is independently associated with LV diastolic dysfunction in young adult patients with SLE., (© 2013 Wiley Periodicals, Inc.)

Aortic atherosclerosis (AoA) defined as intima-media thickening or plaques and aortic stiffness (AoS) also considered an atherosclerotic process and defined as decreased vessel distensibility (higher pulse pressure to achieve similar degree of vessel distension) are common in patients with SLE. Immune-mediated inflammation, thrombogenesis, traditional atherogenic factors, and therapy-related metabolic abnormalities are the main pathogenic factors of AoA and AoS. Pathology of AoA and AoS suggests an initial subclinical endothelialitis or vasculitis, which is exacerbated by thrombogenesis and atherogenic factors and ultimately resulting in AoA and AoS. Computed tomography (CT) for detection of arterial wall calcifications and arterial tonometry for detection of increased arterial pulse wave velocity are the most common diagnostic methods for detecting AoA and AoS, respectively. MRI may become a more applicable and accurate technique than CT. Although transesophageal echocardiography accurately detects earlier and advanced stages of AoA and AoS, it is semi-invasive and cannot be used as a screening method. Although imaging techniques demonstrate highly variable prevalence rates, on average about one third of adult SLE patients may have AoA or AoS. Age at SLE diagnosis; SLE duration; activity and damage; corticosteroid therapy; metabolic syndrome; chronic kidney disease; and mitral annular calcification are common independent predictors of AoA and AoS. Also, AoA and AoS are highly associated with carotid and coronary atherosclerosis. Earlier stages of AoA and AoS are usually subclinical. However, earlier stages of disease may be causally related or contribute to peripheral or cerebral embolism, pre-hypertension and hypertension, and increased left ventricular afterload resulting in left ventricular hypertrophy and diastolic dysfunction. Later stages of disease predisposes to visceral ischemia, aortic aneurysms and aortic dissection. Even earlier stages of AoA and AoS have been associated with increased cardiovascular and cerebrovascular morbidity and mortality of SLE patients. Aggressive non-steroidal immunosuppressive therapy and non-pharmacologic and pharmacologic interventions for control of atherogenic risk factors may prevent the development or progression of AoA and AoS and may decrease cardiovascular and cerebrovascular morbidity and mortality in SLE.

Objectives: The aim of this study was to determine whether Libman-Sacks endocarditis is a pathogenic factor for cerebrovascular disease (CVD) in systemic lupus erythematosus (SLE)., Background: A cardioembolic pathogenesis of SLE CVD manifested as: 1) neuropsychiatric systemic lupus erythematosus (NPSLE), including stroke and transient ischemic attacks (TIA); 2) neurocognitive dysfunction; and 3) magnetic resonance imaging of focal brain lesions has not been established., Methods: A 6-year study of 30 patients with acute NPSLE (27 women, 38 ± 12 years of age), 46 age- and sex-matched SLE controls without NPSLE (42 women, 36 ± 12 years of age), and 26 age- and sex-matched healthy controls (22 women, 34 ± 11 years of age) who underwent clinical and laboratory evaluations, transesophageal echocardiography, carotid duplex ultrasound, transcranial Doppler ultrasound, neurocognitive testing, and brain magnetic resonance imaging/magnetic resonance angiography. Patients with NPSLE were re-evaluated after 4.5 months of therapy. All patients were followed clinically for a median of 52 months., Results: Libman-Sacks vegetations (87%), cerebromicroembolism (27% with 2.5 times more events per hour), neurocognitive dysfunction (60%), and cerebral infarcts (47%) were more common in NPSLE than in SLE (28%, 20%, 33%, and 0%) and healthy controls (8%, 0%, 4%, and 0%, respectively) (all p ≤ 0.009). Patients with vegetations had 3 times more cerebromicroemboli per hour, lower cerebral blood flow, more strokes/TIA and overall NPSLE events, neurocognitive dysfunction, cerebral infarcts, and brain lesion load than those without (all p ≤ 0.01). Libman-Sacks vegetations were independent risk factors of NPSLE (odds ratio [OR]: 13.4; p < 0.001), neurocognitive dysfunction (OR: 8.0; p = 0.01), brain lesions (OR: 5.6; p = 0.004), and all 3 outcomes combined (OR: 7.5; p < 0.001). Follow-up re-evaluations in 18 of 23 (78%) surviving patients with NPSLE demonstrated improvement of vegetations, microembolism, brain perfusion, neurocognitive dysfunction, and lesion load (all p ≤ 0.04). Finally, patients with vegetations had reduced event-free survival time to stroke/TIA, cognitive disability, or death (p = 0.007)., Conclusions: The presence of Libman-Sacks endocarditis in patients with SLE was associated with a higher risk for embolic CVD. This suggests that Libman-Sacks endocarditis may be a source of cerebral emboli., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Aim: Phosphatidylserine-rich microparticles derived from endothelial cells, platelets and leukocytes have been implicated as surrogate markers of cellular activation in systemic lupus erythematosus (SLE). Because microparticles have also been associated with many primary neurologic diseases, this study investigated whether cellular-derived microparticles are also implicated in neuropsychiatric SLE (NPSLE)., Method: Plasma microparticles were measured in 51 SLE patients and 22 age- and gender-matched controls. Acute NPSLE was defined as major NPSLE (acute stroke, transient ischemic attack, psychosis, isolated seizures, major cognitive disorder, or acute confusional state) and NPSLE disease activity was measured with the neurologic components of the SLE Disease Activity Index (Neuro-SLEDAI)., Results: Neuro-SLEDAI levels varied widely in SLE patients, consistent with variable NPSLE activity. When considering all patients with SLE, there was no difference in total microparticles relative to matched controls, 2158/μL (interquartile range [IQR] 1214-3463) versus 2782/μL (IQR 1586-2990; P = 0.57) nor differences in microparticles derived from either platelets (P = 0.40), monocytes (P = 0.15) or endothelial cells (P = 0.32). However, levels of circulating monocyte-derived microparticles significantly and independently correlated with NPSLE (r = -0.28; P = 0.045), corticosteroid dosage (r = -0.38; P = 0.006) and levels of circulating C5a (r = 0.54; P < 0.0001). Non-neurologic SLE disease activity was not associated with microparticles., Conclusion: Circulating cell-derived microparticles are reduced in active NPSLE, although the relative contribution of reduced microparticle production, increased consumption or intravascular sequestration, remain uncertain., (© 2013 The Authors International Journal of Rheumatic Diseases © 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.)

Objective: In previous studies cerebral blood flow (CBF) was found to be altered in patients with systemic lupus erythematosus (SLE) compared to controls. We investigated the relationships between CBF and clinical data from subjects with SLE with the aim of determining the pathologic factors underlying altered CBF in SLE., Methods: A total of 42 SLE subjects and 19 age- and sex-matched healthy control subjects were studied. Dynamic susceptibility contrast (DSC) magnetic resonance imaging (MRI) was used to measure CBF. Patients and controls underwent complete clinical and laboratory evaluations in close proximity with their MRI studies., Results: A higher CBF was present in the SLE group and was independently associated in statistical models with higher systolic blood pressure (SBP; p < 0.01). The intensity of the relationships (slope of curve) between CBF and mean arterial blood pressure, diastolic blood pressure, or blood levels of tissue plasminogen activator in the SLE group was significantly blunted relative to the control group., Conclusion: These findings are consistent with an underlying cerebral hyperperfusion in SLE induced by elevated but nonhypertensive levels of SBP. The factors underlying this relationship may be functional and/or structural (atherosclerotic, thrombotic, thromboembolic, or vasculitic) cerebrovascular disease.

We present images observed in a patient with methicillin-resistant Staphylococcus aureus bacteremia from a hemodyalisis catheter and demonstrate the superiority of real time three-dimensional transesophageal echocardiography (RT3D-TEE) over 2D-TEE at identifying catheter-associated infected vegetations. Other studies have reported RT3D-TEE as an improved modality to identify and characterize intracardiac structures. However, to the best of our knowledge, there are no reports describing the specific characteristics of bacterial vegetations in a dialysis catheter. RT3D-TEE could have a central role in the diagnosis of catheter-related blood stream infections, especially given the increased number of hemodyalisis-dependent patients and increased incidence of infections., (© 2012, Wiley Periodicals, Inc.)

Patients diagnosed with Systemic Lupus Erythematosus have similar levels of neuropsychological dysfunction (i.e., 20-50%) as those with Neuropsychiatric Systemic Lupus Erythematosus (NPSLE). We hypothesized a gradient between cognition and white matter integrity, such that strongest brain-behavior relationships would emerge in NPSLE, intermediate in non-NPSLE, and minimal in controls. We studied thirty-one patients (16 non-NPSLE; 15 NPSLE), ranging in age from 18 to 59 years old (100% female), and eighteen age and gender matched healthy controls. DTI examinations were performed on a 1.5T scanner. A broad neuropsychological battery was administered, tapping attention, memory, processing speed, and executive functioning. The Total z-score consisted of the combined sum of all neuropsychological measures. In control subjects, we found no significant FA-Total z-score correlations. NPSLE, non-NPSLE, and control subjects differed significantly in terms of Total z-score (NPSLE = -2.25+/-1.77, non-NPSLE = -1.22+/-1.03, Controls = -0.10+/-.57; F = 13.2, p<.001). In non-NPSLE subjects, FA within the right external capsule was significantly correlated with Total z-score. In NPSLE subjects, the largest FA-Total z-score clusters were observed within the left anterior thalamic radiation and right superior longitudinal fasciculus. In subsequent analyses the largest number of significant voxels linked FA with the Processing Speed z-score in NPSLE. The current results reflect objective white matter correlates of neuropsychological dysfunction in both NPSLE and (to a lesser degree) in non-NPSLE. non-NPSLE and NPSLE subjects did not differ significantly in terms of depression, as measured by the GDI; thus, previous hypotheses suggesting moderating effects of depression upon neuropsychological performance do not impact the current FA results.

Objective: Magnetic resonance imaging (MRI) often demonstrates brain lesions in neuropsychiatric systemic lupus erythematosus (NPSLE). The present study compared postmortem histopathology with premortem MRI in NPSLE., Methods: Two hundred subjects with NPSLE were studied prospectively with MRI over a 10-year period during which 22 subjects died. In 14 subjects, a brain autopsy with histopathology, that permitted direct comparison with premortem MRI, was successfully obtained. Surface anatomy was used to determine the approximate location of individual lesions., Results: Premortem MRI findings in fatal NPSLE were small focal white matter lesions (100%), cortical atrophy (64%), ventricular dilation (57%), cerebral edema (50%), diffuse white matter abnormalities (43%), focal atrophy (36%), cerebral infarction (29%), acute leukoencephalopathy (25%), intracranial hemorrhage (21%), and calcifications (7%). Microscopic findings in fatal NPSLE included global ischemic changes (57%), parenchymal edema (50%), microhemorrhages (43%), glial hyperplasia (43%), diffuse neuronal/axonal loss (36%), resolved cerebral infarction (33%), microthomboemboli (29%), blood vessel remodeling (29%), acute cerebral infarction (14%), acute macrohemorrhages (14%), and resolved intracranial hemorrhages (7%). Cortical atrophy and ventricular dilation seen by MRI accurately predicted brain mass at autopsy (r = -0.72, P = 0.01, and r = -0.77, P = 0.01, respectively). Cerebral autopsy findings, including infarction, cerebral edema, intracranial hemorrhage, calcifications, cysts, and focal atrophy, were also predicted accurately by premortem MRI., Conclusion: Brain lesions in NPSLE detected by MRI accurately represent serious underlying cerebrovascular and parenchymal brain injury on pathology., (Copyright 2010 Elsevier Inc. All rights reserved.)

Background: Neuropsychiatric systemic lupus erythematosus (NPSLE) is associated with increased morbidity and mortality., Methods: We used Diffusion Tensor Imaging (DTI) to assess white matter abnormalities in seventeen NPSLE patients, sixteen SLE patients without NPSLE, and twenty age- and gender-matched controls., Results: NPSLE patients differed significantly from SLE and control patients in white matter integrity of the body of the corpus callosum, the left arm of the forceps major and the left anterior corona radiata., Conclusions: Several possible mechanisms of white matter injury are explored, including vascular injury, medication effects, and platelet or fibrin macro- or microembolism from Libman-Sacks endocarditis.

Objective: To determine the histopathologic basis of altered brain neurometabolites in neuropsychiatric systemic lupus erythematosus (NPSLE)., Methods: Brain neurometabolite concentrations in a 20-voxel area of the brain were determined premortem by magnetic resonance spectroscopy (MRS) in 7 individuals with NPSLE. Absolute concentrations of neurometabolite for N-acetylaspartate (NAA), choline, creatine, and lactate were measured. After the death of the patients, histopathologic changes were determined at autopsy of the brain and were matched voxel-by-voxel with the neurometabolites., Results: The mean +/- SD absolute concentrations of NAA (9.15 +/- 1.78 mM in patients versus 12.2 +/- 0.8 mM in controls; P < 0.01) and creatine (6.43 +/- 0.16 mM in patients versus 6.90 +/- 0.60 mM in controls; P < 0.003) were significantly reduced and the concentration of choline (2.51 +/- 0.42 mM in patients versus 1.92 +/- 0.32 mM in controls; P < 0.04) was significantly elevated in NPSLE patients as compared with controls. Widespread heterogeneous changes in the histologic features of the brain were present, including microinfarcts, microhemorrhages, bland angiopathy, thrombotic angiopathy with platelet and fibrin thrombi, neuronal necrosis in various states of resolution, reduced numbers of axons and neurons, vacuole and space formation among the fibers, reduced numbers of oligodendrocytes, reactive microglia and astrocytes, lipid-laden macrophages, and cyst formation. Neurometabolite abnormalities were closely associated with underlying histopathologic changes in the brain: 1) elevated choline levels were independently associated with gliosis, vasculopathy, and edema (r = 0.75, P < 0.004 in the multivariate model); 2) reduced creatine levels with reduced neuronal-axonal density and gliosis (r = 0.72, P < 0.002 in the multivariate model); 3) reduced NAA levels with reduced neuronal-axonal density (r = 0.66, P < 0.001 in the multivariate model); and 4) the presence of lactate with necrosis, microhemorrhages, and edema (r = 0.996, P < 0.0001 in the multivariate model)., Conclusion: Altered neurometabolites in NPSLE patients, as determined by MRS, are a grave prognostic sign, indicating serious underlying histologic brain injury.

Within systemic lupus erythematosus (SLE) patients can be divided into groups with and without central nervous system involvement, the latter being subcategorized as neuropsychiatric systemic lupus erythematosus (NPSLE). While a number of research groups have investigated NPSLE, there remains a lack of consistent application of this diagnostic criteria within neuroimaging studies. Previous neuroimaging research suggests that SLE patients have reduced subcortical and regional gray matter volumes when compared to controls, and that these group differences may be driven by SLE patients with neuropsychiatric symptoms. The current study sought to compare measures of cortical thickness and subcortical structure volume between NPSLE, SLE, and healthy controls. We hypothesized that patients with NPSLE (N = 21) would have thinner cortex and reduced subcortical gray matter volumes when compared to SLE (N = 16) and control subjects (N = 21). All subjects underwent MRI examinations on a 1.5 Tesla Siemens Sonata scanner. Anatomical reconstruction and segmentation were performed using the FreeSurfer image analysis suite. Cortical and subcortical volumes were extracted from FreeSurfer and analyzed for group differences, controlling for age. The NPSLE group exhibited decreased cortical thickness in clusters of the left frontal and parietal lobes as well as in the right parietal and occipital lobes compared to control subjects. Compared to the SLE group, the NPSLE group exhibited comparable thinning in clusters of the frontal and temporal lobes. Controlling for age, we found that between group effects for subcortical gray matter structures were significant for the thalamus (F = 3.06, p = .04), caudate nucleus (F = 3.19, p = .03), and putamen (F = 4.82, p = .005). These results clarify previous imaging work identifying cortical atrophy in a mixed SLE and NPSLE group, and suggest that neuroanatomical abnormalities are specific to SLE patients diagnosed with neuropsychiatric symptoms. Future work should help elucidate the underlying mechanisms underlying the emerging neurobiological profile seen in NPSLE, as well as clarify the apparent lack of overlap between cortical thinning and functional activation results and other findings pointing to increased functional activation during cognitive tasks.

Objective: Premature carotid and coronary atherosclerosis are common in systemic lupus erythematosus (SLE), but data on aortic atherosclerosis (AA) are limited. Thus, using multiplane transesophageal echocardiography (TEE), we sought to determine the prevalence and clinical correlates of AA in patients with SLE., Methods: Forty-seven patients with SLE (44 women, age 38 +/- 12 years) and 21 healthy controls (19 women, age 34 +/- 12 years) underwent clinical and laboratory evaluations and TEE to assess AA defined as aortic intima media thickness (IMT) > 0.86 mm or plaques as > 50% focal IMT as compared with surrounding walls. TEE studies were interpreted by an experienced observer unaware of subjects' clinical data., Results: The prevalence of abnormal aortic IMT, plaques, or both lesions was higher in patients as compared to controls (37%, 23%, and 43% vs 14%, 0%, and 14%, respectively, all p

Libman-Sacks endocarditis is the most common cardiac manifestation in patients with systemic lupus erythematosus (SLE) and primary antiphospholipid syndrome (PAPS); it is characterized by Libman-Sacks vegetations, valve thickening or fibrosis, valve regurgitation, and rarely valve stenosis. It is most commonly clinically manifested with cardioembolism (predominantly to the brain), uncommonly with symptomatic severe valve regurgitation, or rarely with over imposed infective endocarditis. These three clinical syndromes are associated with a five to eight times higher morbidity and mortality than in a general population. Libman-Sacks endocarditis is infrequently detected by the history and cardiovascular physical examination; it is detected by transthoracic echocardiography (TTE) in 30-40% as compared to 60-80% of patients by transesophageal echocardiography (TEE). Also, TTE as compared to TEE has low sensitivity (63% overall, 11% for valve vegetations), low specificity (58%), low negative predictive value (40%), and a moderate positive predictive value (78%) for the detection of Libman-Sacks endocarditis. In addition, TEE has played a major role in our current understanding of the specific characteristics, evolution, assessment of prognosis, and defining the need and response to medical or surgical therapy of Libman-Sacks endocarditis. Therefore, an increased awareness of the potential clinical complications of Libman-Sacks endocarditis and application of echocardiography, especially of TEE, may lead to an earlier and accurate diagnosis, guidance of therapy, prevention of complications, decreased rate of progression of the disease, and consequently to an event free survival of patients with SLE or PAPS.

Systemic lupus erythematosus (SLE) is a prototypical autoimmune disease that is atherogenic. Decreased arterial distensibility (AD) is a risk factor for cardiovascular disease, and this precursor may be associated with SLE. Accordingly, we tested the hypothesis that patients with SLE will have significantly (p < 0.05) decreased AD when compared to normal, healthy age, and gender matched controls. Noninvasive, high resolution ultrasound was performed on 30 patients with chronic SLE and 16 age and gender matched controls. All were female. Maximum systolic and minimum diastolic diameters (mm) and intima-media thickness (IMT, mm) in the right common carotid artery were measured from M-mode images. In vitro arterial models were used for quality control. With a single, blinded observer, the 95% confidence levels for accuracy and precision for noninvasive systolic and diastolic tonometric arm blood pressures (SBP, DBP) and carotid sonographic diameters were approximately 5 mmHg and approximately 0.10 mm, respectively. Derived measurements for strain (%), stiffness (units), and AD (units) were determined by published arterial mechanical models and algorithms. Results (mean/standard deviation) were as follows: (patients/controls; # =p<0.05) Age 39/11, 35/11 years; SBP 130/20, 117/8# mmHg; DBP 82/11, 74/9# mmHg; strain 11/4, 11/4 %); stiffness 19/10, 17/11 units; IMT 0.44/0.08, 0.41/0.06 mm; AD 3.10/1.49, 3.30/1.63 units. There were no statistically significant differences (p<0.05) in measurements of AD and IMT in the common carotid artery between relatively young SLE patients and well matched controls.

Pericarditis was the primary manifestation of aortic dissection in these 2 young men. Both patients had no phenotypic characteristics of Marfan or Ehlers-Danlos syndrome. These patients had pleuritic chest pain and characteristic electrocardiographic changes consistent with pericarditis. However, timely performed transthoracic echocardiograms revealed proximal aortic dissection with hemopericardium noted at surgery in both cases. Although the sensitivity of transthoracic echocardiogram for proximal aortic dissection is approximately 60%, certain findings can alert the physician to the possibility of aortic dissection. Therefore, in young patients with suspected pericarditis, a timely performed transthoracic echocardiogram should include a careful evaluation of the ascending aorta and arch to rule out this lethal diagnosis.

Valvular heart disease (VHD) associated with rheumatoid arthritis (RA) has not been well characterized and its clinical predictors are undefined. Therefore, 34 volunteers with RA with a mean age of 50 +/- 10 years underwent clinical evaluation and transesophageal echocardiography. Findings on transesophageal echocardiography were compared with those of 34 gender-matched healthy volunteers with a mean age of 42 +/- 6 years. Twenty patients (59%) had mainly (97%) left-sided VHD (valve nodules in 11, 32%; valve thickening in 18, 53%; valve regurgitation in 7, 21%; and valve stenosis in 1, 3%) compared with 5 controls (15%; [nodules in 1, 3%; thickening in 4, 12%; and regurgitation in 1, 3%; p < or =0.05 for all vs patients). Valve nodules were generally single and small (4 to 12 mm); were oval with regular borders and had homogenous echocardiographic reflectance; were typically located at the leaflets' basal or mid portions; and equally affected the aortic and mitral valves. Valve thickening was equally diffuse or localized; when localized affected any leaflet portion; was usually mild (89%); involved similarly the mitral and aortic valves (47% and 32%, respectively); and rarely (6%) involved the annulus and subvalvular apparatus. Valve regurgitation manifested as mild aortic regurgitation in 4 patients, moderate mitral regurgitation in 4 patients, and moderate tricuspid regurgitation in 1 patient. Mitral and aortic valve stenoses occurred in 1 patient (3%). No correlation was found between VHD and duration, activity, severity, pattern of onset and course, extra-articular disease, serology, or therapy of RA. In conclusion, RA-associated VHD is common, valve nodules and thickening are its distinctive features, and it is not associated with clinical variables of RA.

Background: Previous studies using transesophageal echocardiography (TEE) report an association of valvular heart disease (VHD) with cerebral infarcts and central neuropsychiatric systemic lupus erythematosus (NPSLE). However, TEE cannot be routinely used., Aim: To determine if VHD detected by transthoracic echocardiography (TTE) is associated with focal brain injury on magnetic resonance imaging (MRI) and secondarily with central NPSLE., Methods: Sixty-nine patients with systemic lupus erythematosus underwent general clinical, neuropsychiatric and laboratory evaluations followed by MRI of the brain and TTE., Results: Forty-one patients (59%) had NPSLE (stroke, transient ischemic attack, cognitive dysfunction, acute confusional state, seizures or psychosis); 46 (67%) had focal brain injury on MRI (cerebral infarcts, white matter lesions or small punctate lesions); 38 (55%) had VHD (vegetations, thickening or regurgitation). VHD was more common in patients with than in those without focal brain injury and NPSLE (all p < 0.05); focal brain lesions were more common in patients with than in those without NPSLE (all p < 0.04); and VHD was an independent predictor of focal brain lesions and NPSLE (both p < 0.04)., Conclusion: In patients with systemic lupus erythematosus, VHD detected by TTE is associated with focal brain injury and NPSLE., ((c) 2007 S. Karger AG, Basel.)