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Sex is an important factor associated with pulmonary embolism (PE) disease presentation and outcomes, which may be related to pathobiological, social, and treatment-based differences. We are seeking to illuminate sex differences in pulmonary embolism presentation, care, and outcomes using an international registry and a national US database of people 65 years and older, the age group in which the majority of these events occur.The Sex Differences in PrEsentation, Risk Factors, Drug and Interventional Therapies, and OUtcomes of Elderly PatientS with Pulmonary Embolism (SERIOUS-PE) study has been designed to address knowledge gaps in this area. This study will use data from the Registro Informatizado Enfermedad TromboEmbolica (RIETE) registry and the US Medicare Fee-For-Service beneficiaries. RIETE is a large international registry of patients with venous thromboembolism with data collected on PE presentation, risk factors, co-morbidities, drug and interventional therapies, as well as 30-day and 1-year outcomes (including recurrent VTE, major bleeding, and mortality). Data from US Medicare Fee-For-Service beneficiaries will be used to understand the sex differences in PE hospitalizations, advanced therapies, and outcomes at 30-day and 1-year follow-up. Assessment of outcomes in both databases will be performed in unadjusted models, as well as those adjusted for demographics, co-morbidities, and treatments, to understand whether the potential sex differences in outcomes are related to differences in risk factors and co-morbidities, potential disparities in treatment, or a plausible biological difference in women versus men. Linear trends will be assessed over time.RIETE data from March 2001 through March 2021 include 33,462 elderly patients with PE, of whom 19,294 (57.7%) were women and 14,168 (42.3%) were men. In the Medicare Fee-For-Service database, between January 2001 and December 31, 2019, 1,030,247 patients were hospitalized with a principal discharge diagnosis of PE, of whom 599,816 (58.2%) were women and 430,431 (41.8%) were men.Findings from the SERIOUS-PE study will help address important knowledge gaps related to sex differences in presentation and risk factors, treatment patterns, and outcomes of older adults with PE. The results may guide changes in prognostic prediction rules based on sex-specific findings, identify sex-based disparities in care delivery that should be addressed by quality improvement, or uncover potential differences in response to available therapies that warrant testing in dedicated randomized trials.

(1) describe imaging features of CIA, (2) compare dilation rate and wall thickening of aortic aneurysms in patients with CIA versus those with giant cell arteritis/aortitis (GCA), (3) present clinical outcomes of CIA patients. Retrospective search of electronic records from 2004 to 2018 yielded 71 patients, 52 of whom were female, with a mean age of 67.5 ± 9.0 years old, with a new clinical diagnosis of cranial or extracranial GCA (GCA group), and giant cell aortitis revealed by the aortic biopsy (CIA group). Comparisons between groups were conducted using the Wilcoxon rank-sum and Fisher’s exact tests. Survival from the date of initial diagnosis to the end of data collection was compared between the two groups through a log-rank test. CIA patients (n = 23; 32%) presented with cardiovascular symptoms, and none had systemic inflammatory symptoms. Inflammatory markers were significantly higher among GCA patients than among CIA patients (p < 0.0001). The CIA group demonstrated thoracic aortic aneurysms without wall thickening. None of the GCA patients (n = 48; 68%) had aneurysmal dilation in the aorta at the time of diagnosis. None of the four CIA patients had FDG uptake in the aorta, while nine out of 13 GCA patients had FDG uptake in the vessels. There was no statistically significant difference in the survival between the two groups (p = 0.12). CIA patients presented with cardiovascular symptoms and was characterized by aneurysm of the aorta without the involvement of the infrarenal aortic segment. The role of FDG-PET/CT in CIA is less certain, though none of the patients in this cohort had FDG uptake in the vessels.

International audience; This expert consensus statement on the interpretation of peripheral arterial and venous spectral Doppler waveforms was jointly commissioned by the Society for Vascular Medicine (SVM) and the Society for Vascular Ultrasound (SVU). The consensus statement proposes a standardized nomenclature for arterial and venous spectral Doppler waveforms using a framework of key major descriptors and additional modifier terms. These key major descriptors and additional modifier terms are presented alongside representative Doppler waveforms, and nomenclature tables provide context by listing previous alternate terms to be replaced by the new major descriptors and modifiers. Finally, the document reviews Doppler waveform alterations with physiologic changes and disease states, provides optimization techniques for waveform acquisition and display, and provides practical guidance for incorporating the proposed nomenclature into the final interpretation report.

Objective Coronavirus disease 2019 (COVID-19) is associated with abnormal inflammatory and coagulation markers, potentially mediating thrombotic events. Our objective was to investigate the incidence, time course, laboratory features, and in-hospital outcomes of COVID-19 patients with suspected venous thromboembolism (VTE). Methods A retrospective observational cohort study was conducted of patients hospitalized with COVID-19 who had undergone ultrasound imaging for suspected VTE from March 13 to May 18, 2020. The medical records of the included patients were reviewed for D-dimer, fibrinogen, prothrombin time, partial thromboplastin time, platelet count, C-reactive protein (CRP), and high-sensitivity troponin T at admission and at up to seven time points before and after ultrasound examination. The clinical outcomes included superficial venous thrombosis, deep vein thrombosis, pulmonary embolism, intubation, and death. Mixed effects logistic, linear, and Cox proportional hazards methods were used to evaluate the relationships between the laboratory markers and VTE and other in-hospital outcomes. Results Of 138 patients who had undergone imaging studies, 44 (31.9%) had evidence of VTE. On univariable analysis, an elevated admission CRP (odds ratio [OR], 1.05; 95% confidence interval [CI], 1.01-1.09; P = .02; per 10-U increase in CRP), platelet count (OR, 1.48; 95% CI, 1.04-2.12; P = .03; per 1000-U increase in platelet count), and male sex (OR, 2.64; 95% CI, 1.19-5.84; P = .02), were associated with VTE. However only male sex remained significant on multivariable analysis (OR, 2.37; 95% CI, 1.01-5.56; P = .048). The independent predictors of death included older age (hazard ratio [HR], 1.04; 95% CI, 1.00-1.07; P = .04), active malignancy (HR, 4.39; 95% CI, 1.39-13.91; P = .01), elevated admission D-dimer (HR, 1.016; 95% CI, 1.003-1.029; P = .02), and evidence of disseminated intravascular coagulation (HR, 4.81; 95% CI, 1.76-13.10; P = .002). Conclusions Male sex, elevated CRP, and elevated platelet count at admission were associated with VTE on univariable analysis. However, only male sex remained significant on multivariable analysis. Older age, active malignancy, disseminated intravascular coagulation, and elevated D-dimer at admission were independently associated with death for patients hospitalized with COVID-19.

Peripheral artery disease is a prevalent but underdiagnosed manifestation of atherosclerosis. There is insufficient awareness of its clinical manifestations, including intermittent claudication and critical limb ischemia and of its risk of adverse cardiovascular and limb outcomes. In addition, our inadequate knowledge of its pathophysiology has also limited the development of effective treatments, particularly in the presence of critical limb ischemia. This review aims to highlight essential elements of the epidemiology and pathophysiology of peripheral artery disease, bring attention to the often-atypical manifestations of occlusive arterial disease of the lower extremity, increase awareness of critical limb ischemia, briefly describe the diagnostic role of the ankle brachial index, and go over the contemporary management of peripheral artery disease. An emphasis is placed on evidence-based medical treatments to improve symptoms and quality of life and to reduce the risk of cardiovascular and limb events in these patients, including supervised exercise training, smoking cessation, antagonism of the renin-angiotensin system, lipid-lowering, antiplatelet, and antithrombotic therapies.

A young woman with mandibuloacral dysplasia, a syndrome on the progeria spectrum with accelerated vascular calcification and calcific valve stenosis, presented with symptomatic severe aortic stenosis. She underwent transcatheter aortic valve replacement with a balloon-expandable valve, and her exertional symptoms improved significantly. (Level of Difficulty: Intermediate.), Central Illustration

Purpose: Central retinal artery occlusion (CRAO) is a form of acute ischemic stroke that causes severe visual loss and is a harbinger of further cerebrovascular and cardiovascular events. There is a paucity of scientific information on the appropriate management of CRAO, with most strategies based on observational literature and expert opinion. In this scientific statement, we critically appraise the literature on CRAO and provide a framework within which to consider acute treatment and secondary prevention. Methods: We performed a literature review of randomized controlled clinical trials, prospective and retrospective cohort studies, case-control studies, case reports, clinical guidelines, review articles, basic science articles, and editorials concerning the management of CRAO. We assembled a panel comprising experts in the fields of vascular neurology, neuro-ophthalmology, vitreo-retinal surgery, immunology, endovascular neurosurgery, and cardiology, and document sections were divided among the writing group members. Each member received an assignment to perform a literature review, synthesize the data, and offer considerations for practice. Multiple drafts were circulated among the group until consensus was achieved. Results: Acute CRAO is a medical emergency. Systems of care should evolve to prioritize early recognition and triage of CRAO to emergency medical attention. There is considerable variability in management patterns among practitioners, institutions, and subspecialty groups. The current literature suggests that treatment with intravenous tissue plasminogen activator may be effective. Patients should undergo urgent screening and treatment of vascular risk factors. There is a need for high-quality, randomized clinical trials in this field.

Background: Segmental arterial mediolsyis (SAM) is a non-inflammatory arteriopathy that is increasingly being recognized; however, its clinical characteristics and natural history remain poorly defined. Methods: A retrospective, single-institution review of 20 patients presenting with arterial dissection, intramural hematoma, aneurysm, or occlusion between 2015 and 2020 was performed. To establish a non-invasive diagnosis of SAM, patients with FMD, inflammatory, or genetic arteriopathy were excluded by clinical, laboratory, and imaging criteria according to multidisciplinary guidelines. Patient demographics, clinical features, imaging findings, and management were assessed. Results: The average age of patients was 56 years; 75% were male. CAD was present in 15% of patients and 45% had hypertension; 55% were current or prior smokers. In patients without diagnosed CAD, the 10-year ASCVD risk score was 8.7% and the Framingham risk score was 6.8%. The average hemoglobin A1c was 5.6%. Acute onset abdominal pain (70%) was the most frequent presenting symptom. On average, two different arterial beds were affected at the time of diagnosis, most often in the abdomen. Arterial dissection was present in 75% of patients, and intramural hematoma was seen in 35% of cases. The most affected vessel was the superior mesenteric artery (50%), followed by the celiac, renal, and iliac arteries (35% each). Over a mean follow-up period of 20 months, all patients survived; two patients required intervention due to worsening clinical symptoms. Anticoagulation (AC) was utilized in 50% of cases, most commonly for one month. Long-term follow-up imaging was available in 11 patients; 3 patients (27%) had progression in lesion size over the first month. By 1 year, only 1 patient continued to show enlargement, with all others showing regression. Conclusions: This cohort provides longitudinal follow-up on both clinical and imaging characteristics of non-invasively diagnosed SAM. The condition shows a male predominance, typically manifesting in middle-aged patients with low to intermediate cardiovascular risk. While a subset of patients shows growth in size of the affected vascular segment over the first month, longer follow-up imaging demonstrates regression.

(1) describe imaging features of CIA, (2) compare dilation rate and wall thickening of aortic aneurysms in patients with CIA versus those with giant cell arteritis/aortitis (GCA), (3) present clinical outcomes of CIA patients. Retrospective search of electronic records from 2004 to 2018 yielded 71 patients, 52 of whom were female, with a mean age of 67.5 ± 9.0 years old, with a new clinical diagnosis of cranial or extracranial GCA (GCA group), and giant cell aortitis revealed by the aortic biopsy (CIA group). Comparisons between groups were conducted using the Wilcoxon rank-sum and Fisher's exact tests. Survival from the date of initial diagnosis to the end of data collection was compared between the two groups through a log-rank test. CIA patients (n = 23; 32%) presented with cardiovascular symptoms, and none had systemic inflammatory symptoms. Inflammatory markers were significantly higher among GCA patients than among CIA patients (p0.0001). The CIA group demonstrated thoracic aortic aneurysms without wall thickening. None of the GCA patients (n = 48; 68%) had aneurysmal dilation in the aorta at the time of diagnosis. None of the four CIA patients had FDG uptake in the aorta, while nine out of 13 GCA patients had FDG uptake in the vessels. There was no statistically significant difference in the survival between the two groups (p = 0.12). CIA patients presented with cardiovascular symptoms and was characterized by aneurysm of the aorta without the involvement of the infrarenal aortic segment. The role of FDG-PET/CT in CIA is less certain, though none of the patients in this cohort had FDG uptake in the vessels.

Vascular compression can occur when local anatomy facilitates positional compression of a vessel. Symptoms are determined by the vascular bed supplied by the compressed vessel. These vascular compression syndromes arise in the thorax (thoracic outlet syndrome), abdomen (median arcuate ligament syndrome and nutcracker syndrome) and in the limbs (May-Thurner syndrome and adventitial cyst). While vascular compression is very common, clinical syndromes developing as a result of compression are rare.

Limb swelling can develop suddenly or over the course of years. A correct diagnosis requires consideration of whether the swelling is acute or chronic, symmetric or asymmetric, localized or generalized, and congenital or acquired. Edema develops when the rate of capillary filtration exceeds venous and lymphatic drainage, either because of inadequate lymphatic flow or increased capillary filtration. A careful approach to diagnosis in this setting can allow recognition of diseases requiring urgent treatment as well as allowing appropriate treatment.

There are a number of conditions can impair the blood supply to the kidneys and mesentery, although atherosclerosis is the most common etiology. Additional possibilities include emboli, in situ thrombosis, extrinsic compression, dissection and fibromuscular dysplasia. The first step to diagnosing mesenteric vascular disease is to recognize that there is altered flow to the kidney or mesentery.

The goal of vascular testing is to characterize vessel structure and function. In many cases, a comprehensive history and physical examination are sufficient to make a dignosis (McDermott, Circ Res 116(9):1540–1550, 2015). However, in instances when more information is necessary, the history and physical will direct appropriate diagnostic testing. The modality is chosen according to the needs of a particular patient as well as availability and local expertise. Modalities include physiologic testing, ultrasound (US), computed tomography (CT) and magnetic resonance (MR) imaging (Table 1.1). Specific uses of these methods are discussed further in the disease specific sections of this text. These noninvasive methods are distinct from invasive angiography, where intervention can be performed at the time of diagnosis.

Leg pain is common and there are many etiologies for non-joint leg pain. Vascular, neurologic and musculoskeletal conditions are all important causes of leg pain. It is essential to understand pain character, location, timing and triggers to identify the cause of leg pain. Examination includes an assessment of leg temperature, color, pulses, reflexes, sensation and dynamic maneuvers. Vascular causes such as peripheral artery disease and venous claudication must be considered among these causes of leg pain.

Peripheral artery disease (PAD) is a term referring to obstruction of arterial flow to an end organ, the leg most often being the organ when this term is used. PAD most commonly refers to atherosclerotic disease, and atherosclerotic PAD is most common in those over 65 years of age as well as those with a history of diabetes or cigarette smoking. However, other diseases can obstruct lower extremity arterial flow including arteritis, embolism, fibromuscular dysplasia, and dissection and should be considered especially when the individual with PAD has no atherosclerotic risk factors.

Although the resting ankle–brachial index (ABI) is commonly used as a httptool to diagnose peripheral artery disease (PAD), several additional indices measured after exercise may have increased sensitivity for identifying PAD. The aim of this study was to determine the utility of resting ABI and three post-exercise physiological parameters for diagnosing PAD confirmed by arterial imaging studies. For each qualifying study, we assessed the performance measures for identifying PAD for resting ABI < 0.90, exercise ABI < 0.90, a decrease in ABI > 20% with exercise, and a decrease in ankle pressure > 30 mmHg with exercise. Of the 199 exams that met our inclusion criteria, imaging showed a > 75% stenotic lesion in at least one limb in 138 (69%) of patients. For stenoses > 75%, resting ABI < 0.90 had a sensitivity of 64% (95% CI: 56–72%) and exercise ABI < 0.90 had a sensitivity of 88% (95% CI: 82–93%). The sensitivity for a post-exercise ABI decrease > 20% was 67% (95% CI: 59–75%) and the sensitivity for a decrease in ankle pressure > 30 mmHg was 4% (95% CI: 2–9%). For individuals with a normal resting ABI but stenotic lesions > 75% confirmed by imaging (n=49), the addition of exercise ABI testing correctly identified an additional 25% of this population. Overall, exercise ABI < 0.90 exhibits a greater sensitivity for detecting PAD compared to resting ABI. Furthermore, exercise ABI < 0.90 had added clinical utility in patients with normal resting ABIs and was superior to other commonly used exercise indices.

Abdominal aortic aneurysms (AAAs) are a relatively common vascular problem that can be treated with either open, surgical repair or endovascular aortic aneurysm repair (EVAR). Both approaches to AAA repair require dedicated preoperative imaging to minimize adverse outcomes. After EVAR, cross-sectional imaging has an integral role in confirming the successful treatment of the AAA and early detection of complications related to EVAR. CT angiography is the primary imaging modality for both preoperative planning and follow-up after repair. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Vascular claudication is a symptom complex characterized by reproducible pain and weakness in an active muscle group due to peripheral arterial disease. Noninvasive hemodynamic tests such as the ankle brachial index, toe brachial index, segmental pressures, and pulse volume recordings are considered the first imaging modalities necessary to reliably establish the presence and severity of arterial obstructions. Vascular imaging is consequently used for diagnosing individual lesions and triaging patients for medical, percutaneous, or surgical intervention. Catheter angiography remains the reference standard for imaging the peripheral arteries, providing a dynamic and accurate depiction of the peripheral arteries. It is particularly useful when endovascular intervention is anticipated. When combined with noninvasive hemodynamic tests, however, noninvasive imaging, including ultrasound, CT angiography, and MR angiography, can also reliably confirm or exclude the presence of peripheral arterial disease. All modalities, however, have their own technical limitations when classifying the location, extent, and severity of disease. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer-reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Jonathan L. Halperin, MD, FACC, FAHA, Chair Glenn N. Levine, MD, FACC, FAHA, Chair-Elect Sana M. Al-Khatib, MD, MHS, FACC, FAHA Kim K. Birtcher, PharmD, MS, AACC Biykem Bozkurt, MD, PhD, FACC, FAHA Ralph G. Brindis, MD, MPH, MACC Joaquin E. Cigarroa, MD, FACC Lesley H. Curtis, PhD, FAHA

Background Patients who have undergone intracoronary stent implantation often require surgery within the first year after the procedure. Planned or emergent surgical intervention requires interruption of antiplatelet therapy and is associated with an increased risk of stent thrombosis. Eptifibatide, an intravenous glycoprotein IIb/IIIa inhibitor (GPIIb/IIIa), can be considered for antiplatelet bridging of high-risk patients in the periprocedural period. Objectives The aim of this report is to describe the management of antiplatelet therapy and outcomes of patients who were bridged with eptifibatide perioperatively within 1 year of intracoronary stent implantation. Methods We performed a retrospective analysis of patients identified through the hospital's computer system consecutively from January 1, 2011 to December 31, 2014. We included 18 patients who were bridged from an oral P2Y12-receptor antagonist with eptifibatide before surgery. Outcome measures were the incidence of thromboembolic events or stent thrombosis within 30 days of surgery and death within 90 days of hospital discharge. Safety measures were the incidence of thrombolysis in myocardial infarction major, minor, or minimal bleeding. Results Of the 18 patients assessed, no patients experienced thromboembolic events or stent thrombosis. There was one major bleeding event and one minimal bleeding event postoperatively. Antiplatelet therapy management was highly variable in the perioperative period with 72.2% receiving the recommended GPIIb/IIIa loading dose, 50% of patients not continuing aspirin throughout the surgery, 27.8% of patients stopping antiplatelet therapy less than 5 days before surgery, and 50% not receiving a loading dose of an oral P2Y12-receptor antagonist postoperatively. Conclusions Within a limited sample size, bridging with an intravenous GPIIb/IIIa inhibitor appeared feasible. Further study is needed on the optimal strategy to manage patients with recent stenting who need surgical procedures.

Background: Hutchinson-Gilford progeria syndrome is an extremely rare, fatal, segmental premature aging syndrome caused by a mutation in LMNA yielding the farnesylated aberrant protein progerin. Without progerin-specific treatment, death occurs at an average age of 14.6 years from an accelerated atherosclerosis. A previous single-arm clinical trial demonstrated that the protein farnesyltransferase inhibitor lonafarnib ameliorates some aspects of cardiovascular and bone disease. This present trial sought to further improve disease by additionally inhibiting progerin prenylation. Methods: Thirty-seven participants with Hutchinson-Gilford progeria syndrome received pravastatin, zoledronic acid, and lonafarnib. This combination therapy was evaluated, in addition to descriptive comparisons with the prior lonafarnib monotherapy trial. Results: No participants withdrew because of side effects. Primary outcome success was predefined by improved per-patient rate of weight gain or carotid artery echodensity; 71.0% of participants succeeded ( P P =0.001) and volumetric ( P P P =0.001) and femoral (0% to 12%; P =0.13) artery plaques and extraskeletal calcifications (34.4% to 65.6%; P =0.006) increased. Other than increased bone mineral density, no improvement rates exceeded those of the prior lonafarnib monotherapy treatment trial. Conclusions: Comparisons with lonafarnib monotherapy treatment reveal additional bone mineral density benefit but likely no added cardiovascular benefit with the addition of pravastatin and zoledronic acid. Clinical Trial Registration: URL: http://www.clinicaltrials.gov . Unique identifiers: NCT00879034 and NCT00916747.

Chronic rejection leading to allograft loss remains a significant concern after facial allotransplantation. Chronic rejection may occur without clinical signs or symptoms. The current means of monitoring is histologic analyses of allograft biopsy specimens, which is both invasive and impractical. Prior data suggest that chronic rejection is associated with changes in intima and media thickness of vessels in arms and solid organ allografts; such data have not been published for face transplant recipients.The authors used a 48-MHz transducer to acquire images of the bilateral facial, radial, dorsalis pedis and, if applicable, sentinel flap arteries in five face transplant recipients (8 months to 4.5 years after transplantation) and five control subjects. The authors assessed the intima, media, and adventitia thickness plus lumen and the total vessel diameter and area.Face transplant recipients had thicker intima in all sites compared with controls, but the ratio of the intimal thickness of facial and radial arteries was similar in face transplant recipients compared with controls (1.00 versus 0.95; p = 0.742). Intraobserver correlation showed reliable reproducibility of the measurements (r = 0.935, p ≤ 0.001). Interobserver correlation demonstrated reproducibility of intima measurements (r = 0.422, p ≤ 0.001).The authors demonstrate that ultrasound biomicroscopy is feasible for postsurgical monitoring, and have developed a new benchmark parameter, the facial artery-to-radial artery intimal thickness ratio, to be used in future testing in the setting of chronic rejection.Diagnostic, IV.

Thoracic outlet syndrome is a clinical entity characterized by compression of the neurovascular bundle, and may be associated with additional findings such as venous thrombosis, arterial stenosis, or neurologic symptoms. The goal of imaging is to localize the site of compression, the compressing structure, and the compressed organ or vessel, while excluding common mimics. A literature review is provided of current indications for diagnostic imaging, with discussion of potential limitations and benefits of the respective modalities. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 3 years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment. In this document, we provided guidelines for use of various imaging modalities for assessment of thoracic outlet syndrome.

A 67-year-old woman presented to her primary care physician with leg pain. Seven weeks before presentation she noticed a cramping sensation in the thighs and calves that developed after she had walked a couple of blocks and was relieved with rest.

PURPOSE To evaluate the correlation between the computed tomography (CT)-derived right ventricle (RV) to left ventricle (LV) diameter ratio and the RV size determined by echocardiography in patients with acute pulmonary embolism. MATERIALS AND METHODS Consecutive CT pulmonary angiography examinations (August 2003 to May 2010) from a single, large, urban teaching hospital were retrospectively reviewed. For a cohort of 777 subjects who underwent echocardiography within 48 hours of the CT acquisition, the qualitative RV size (divided into 5 categories) extracted from the echocardiography report was correlated with the CT-derived RV/LV diameter ratio. RESULTS There was moderate correlation (Spearman rank correlation coefficient=0.54, P

Arrhythmia and palpitation are common during pregnancy. Right ventricular outflow tract tachycardia, a rare cause of palpitations occurring even in the absence of structural heart disease, is uncommon during pregnancy. Nevertheless, the presence of right ventricular outflow tract tachycardia in pregnancy requires careful cardiac evaluation with a focus on managing arrhythmogenic activity while maintaining patient comfort and safety. We report a case of right ventricular outflow tract tachycardia in a pregnant 32-year-old woman, whose arrhythmia was detected 2 weeks before labor and persisted through the peripartum period.

Objectives To investigate the pathophysiology of chronic venous insufficiency in adults with a Fontan circulation. Background Chronic venous insufficiency (CVI) is prevalent in adults with a Fontan circulation, however the underlying pathophysiology has not been identified. We defined the prevalence of venous reflux and obstruction in a Fontan population, identified associated factors, and assessed the hypothesis that venous reflux correlates with clinical CVI in adults with a Fontan circulation. Methods Fifty-one adult Fontan subjects from the Congenital Heart Disease in Adults: Lower Extremity Systemic Venous Health in Fontan Patients (CALF) study, ten control subjects without cardiac disease, and ten comparator subjects with repaired tetralogy of Fallot underwent lower extremity Doppler venous ultrasound. We investigated 10 venous segments in each subject for venous obstruction and reflux. Results The prevalence of venous reflux was significantly greater in the Fontan population compared to healthy controls (51% vs. 10%, p=0.03). Venous flow patterns demonstrated a range of pulsatility and biphasic flow in many subjects. There was no evidence of venous obstruction in any subject. Venous reflux did not correlate with clinical venous insufficiency; 49% of Fontan subjects with observed venous reflux demonstrated no clinical signs of venous disease. Predictors of venous reflux in multivariate analysis were single right ventricle (p=0.03), use of anti-arrhythmic medications (p=0.04), and family history of venous disease (p=0.003). Conclusions Venous reflux is highly prevalent and venous obstruction is absent in adults with a Fontan circulation. Clinical CVI underestimates pathophysiologic venous insufficiency in adults with a Fontan circulation.

Mesenteric ischemia is a rare disease associated with high morbidity and mortality. Acute mesenteric ischemia is most commonly secondary to embolism followed by arterial thrombosis, nonocclusive ischemia, and less commonly venous thrombosis. Chronic mesenteric ischemia is almost always caused by atherosclerotic disease, with rare causes including fibromuscular dysplasia and vasculitis. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 2 years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances where evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment. Patients with mesenteric ischemia usually present with nonspecific abdominal symptoms and laboratory findings. This document evaluates and rates the appropriateness of imaging to evaluate patients with clinically suspected mesenteric ischemia. While catheter-based angiography has been considered the reference standard and enables diagnosis and treatment, advances in computed tomography have made it a first-line test in many patients because it is a fast, widely available, and noninvasive study. Abdominal radiographs and ultrasound have a limited role in diagnosing mesenteric ischemia but are commonly the first ordered tests in patients with abdominal pain and may diagnose more common pathologies.

Pulmonary arteriovenous malformations are often included in the differential diagnosis of common clinical presentations, including hypoxemia, hemoptysis, brain abscesses, and paradoxical stroke, as well as affecting 30% to 50% of patients with hereditary hemorrhagic telangiectasia (HHT). Various imaging studies are used in the diagnostic and screening settings, which have been reviewed by the ACR Appropriateness Criteria Vascular Imaging Panel. Pulmonary arteriovenous malformation screening in patients with HHT is commonly performed with transthoracic echocardiographic bubble study, followed by CT for positive cases. Although transthoracic echocardiographic bubble studies and radionuclide perfusion detect right-to-left shunts, they do not provide all of the information needed for treatment planning and may remain positive after embolization. Pulmonary angiography is appropriate for preintervention planning but not as an initial test. MR angiography has a potential role in younger patients with HHT who may require lifelong surveillance, despite lower spatial resolution compared with CT. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every three years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare, fatal, segmental premature aging syndrome caused by a mutation in LMNA that produces the farnesylated aberrant lamin A protein, progerin. This multisystem disorder causes failure to thrive and accelerated atherosclerosis leading to early death. Farnesyltransferase inhibitors have ameliorated disease phenotypes in preclinical studies. Twenty-five patients with HGPS received the farnesyltransferase inhibitor lonafarnib for a minimum of 2 y. Primary outcome success was predefined as a 50% increase over pretherapy in estimated annual rate of weight gain, or change from pretherapy weight loss to statistically significant on-study weight gain. Nine patients experienced a ≥50% increase, six experienced a ≥50% decrease, and 10 remained stable with respect to rate of weight gain. Secondary outcomes included decreases in arterial pulse wave velocity and carotid artery echodensity and increases in skeletal rigidity and sensorineural hearing within patient subgroups. All patients improved in one or more of these outcomes. Results from this clinical treatment trial for children with HGPS provide preliminary evidence that lonafarnib may improve vascular stiffness, bone structure, and audiological status.

Upper-extremity venous thrombosis often presents as unilateral arm swelling. The differential diagnosis includes lesions compressing the veins and causing a functional venous obstruction, venous stenosis, an infection causing edema, obstruction of previously functioning lymphatics, or the absence of sufficient lymphatic channels to ensure effective drainage. The following recommendations are made with the understanding that venous disease, specifically venous thrombosis, is the primary diagnosis to be excluded or confirmed in a patient presenting with unilateral upper-extremity swelling. Contrast venography remains the best reference-standard diagnostic test for suspected upper-extremity acute venous thrombosis and may be needed whenever other noninvasive strategies fail to adequately image the upper-extremity veins. Duplex, color flow, and compression ultrasound have also established a clear role in evaluation of the more peripheral veins that are accessible to sonography. Gadolinium contrast–enhanced MRI is routinely used to evaluate the status of the central veins. Delayed CT venography can often be used to confirm or exclude more central vein venous thrombi, although substantial contrast loads are required. The ACR Appropriateness Criteria ® are evidence-based guidelines for specific clinical conditions that are reviewed every 2 years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.