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BACKGROUND: Anderson-Fabry disease (AFD) is a rare X-linked lysosomal storage disease due to a genetic variation in the α-galactosidase A (GLA) gene. As a result, the activity of the α-galactosidase A (AGAL-A) enzyme is reduced or absent, which causes sphingolipid deposition within different body parts. AFD typically manifests with cardiovascular, renal, cerebrovascular, and dermatologic involvement. Lymphedema is caused by sphingolipid deposition within lymphatics. Lymphedema can cause intolerable pain and limit daily activities. Very limited data exist on lymphedema in AFD patients. METHODS: Using data from the Fabry Registry (NCT00196742) with 7671 patients included (44% males and 56% females), we analyzed the prevalence of lymphedema among AFD patients who were ever assessed for lymphedema and studied the age of first reported lymphedema. Additionally, we assessed whether patients received AFD-specific treatment at some point during their clinical course. The data was stratified by gender and phenotype. RESULTS: Our study showed that lymphedema occurred in 16.5% of the Fabry Registry patients who were ever assessed for lymphedema (n = 5487). Male patients when compared to female patient have higher prevalence (21.7% vs 12.7%) and experienced lymphedema at a younger age (median age at first reported lymphedema of 43.7 vs 51.7 years). When compared to other phenotypes, classic phenotype has the highest prevalence of lymphedema with the earliest reported lymphedema. Among those who reported lymphedema, 84.5% received AFD-specific treatment during their clinical course. CONCLUSIONS: Lymphedema is a common manifestation of AFD in both genders, with a tendency to present later in female patients. Recognition of lymphedema can offer an important opportunity for intervention and potential impact on associated morbidity. Additional future studies are needed to characterize the clinical implications of lymphedema in AFD patients and identify additional treatment options for this growing population.
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Doença de Fabry , Linfedema , Masculino , Feminino , Humanos , Doença de Fabry/complicações , Doença de Fabry/epidemiologia , Doença de Fabry/genética , alfa-Galactosidase/genética , Prevalência , Linfedema/etiologia , Linfedema/genética , Sistema de Registros , Progressão da DoençaRESUMO
PURPOSE OF REVIEW: Management of intracardiac masses, such as right heart thrombi and catheter-related vegetations, can be challenging. Many patients are high-risk candidates for surgical extraction due to multiple comorbidities and risk of distal embolization. In this review, we highlight the advancements in percutaneous approaches for treatment of intracardiac masses utilizing AngioVac (AngioDynamic, Latham, NY). RECENT FINDINGS: With the Food and Drug Administration (FDA) approval of the AngioVac System in 2009, a growing body of evidence has proven it to be a feasible and effective tool to extract thrombi and masses from the iliocaval system and the right heart. In this review, we highlight the feasibility of AngioVac System (AngioDynamic, Latham, NY) based on the published cases series and registries. Future randomized controlled trials are needed to establish an algorithmic approach in treating intracardiac masses.
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Cardiopatias , Trombose , Coração , Cardiopatias/terapia , Humanos , Trombectomia , Resultado do TratamentoRESUMO
PURPOSE OF REVIEW: Heart failure is a complex clinical syndrome with a substantial disease burden. We aim to review the interventional aspects of management of advanced heart failure, focusing on the role of management of coronary artery disease, valvular heart disease, and mechanical circulatory support. RECENT FINDINGS: The patients with coronary artery disease and heart failure requiring revascularization are at higher risk than the rest of the general population. Coronary artery bypass grafting or percutaneous intervention can be used depending on varied patient characteristics and coronary anatomy. Transcatheter aortic valve implantation, Mitraclip and transcatheter ventricular restoration help in tackling the valvular and left ventricular remodeling concerns often seen in this patient population. Temporary hemodynamic circulatory support helps stabilizing patients in cardiogenic shock while long-term support can help bridge them to more definitive therapies. SUMMARY: The management strategies in this disease state are ever evolving with robust evidence coming in support of interventional therapies whenever deemed appropriate. It is the multidisciplinary patient-centered approach, which yields maximum benefit out of these complex interventions.
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Doença da Artéria Coronariana , Insuficiência Cardíaca , Insuficiência Cardíaca/terapia , Coração Auxiliar , Humanos , Choque Cardiogênico , Resultado do TratamentoRESUMO
OBJECTIVE: We sought to examine outcomes of ultrafiltration in real world community-based hospital settings. BACKGROUND: Ultrafiltration (UF) is an accepted therapeutic option for advanced decompensated heart failure (ADHF). the feasibility of UF in a community hospital setting, by general cardiologists in a start-up program had not been objectively evaluated. METHODS: We retrospectively analyzed the first-year cohort of ADHF patients treated with UF from 10/1/2019 to 10/1/2020, which totaled 30 patients, utilizing the CHF Solutions Aquadex FlexFlow™ System with active UF rate titration. RESULTS: Baseline patient characteristics were similar to RCTs: mean age 63, 73 % male; 27 % female; 53 % Caucasian; 47 % African American; 77 % had LVEF ≤ 40. The baseline mean serum creatinine (Cr) was 1.84 ±0.62 mg/dL, mean GFR of 36.95 ±9.60 ml/min. HF re-admission rates were not significantly different than prior studies (17.2 % at 30 d, 23.3 % at 60 d, but in our cohort, per patient HF re-admission rates were reduced significantly by 60 d (0.30 p = 0.017). CONCLUSION: Our analysis showed success with UF in mainstream setting with reproducible results of significant volume loss without adverse renal effect, mitigation of recurrent Hdmissions, and remarkable subjective clinical benefit.
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Etiologies of tricuspid regurgitation are often explored in patients with symptoms of right-sided heart failure. Blunt chest trauma is the major cause of traumatic tricuspid valve regurgitation (TTVR), a secondary type of tricuspid regurgitation. It is a rare condition; however, it may lead to severe consequences if not treated in a timely manner. TTVR should be considered in a patient presenting with chest trauma. In this case, we report a case of a young male who presented after a motor vehicle accident with secondary tricuspid valve regurgitation due to blunt chest trauma as well as a patent foramen ovale.
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Diabetic foot ulcer (DFU) is a debilitating and severe manifestation of uncontrolled and prolonged diabetes that presents as ulceration, usually located on the plantar aspect of the foot. Approximately 15% of individuals with diabetes will eventually develop DFU, and 14%-24% of them will require amputation of the ulcerated foot due to bone infection or other ulcer-related complications. The pathologic mechanisms underlying DFU are comprise a triad: Neuropathy, vascular insufficiency, and secondary infection due to trauma of the foot. Standard local and invasive care along with novel approaches like stem cell therapy pave the way to reduce morbidity, decrease amputations, and prevent mortality from DFU. In this manuscript, we review the current literature with focus on the pathophysiology, preventive options, and definitive management of DFU.
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Sudden cardiac death is a major cause of cardiovascular mortality in the United States with 250,000-450,000 deaths annually. Transvenous Implantable Cardioverter-Defibrillator (ICD) has been conventionally used for both primary and secondary prevention of ventricular tachycardia or fibrillation (VT/VF). It is also associated with a high risk of complications like hemothorax, pneumothorax, cardiac tamponade, lead failure, and increased risk for infections. Subcutaneous ICD (S-ICD) poses as a viable alternative with reduced chances of complications. This manuscript aims to review S-ICD as an alternative to conventional transvenous ICD and its efficacy. We conducted a Medline search of "Subcutaneous," "ICD," "transvenous," and "ventricular tachycardia or fibrillation (VT/VF)" to identify pivotal trials published before June 2021, for inclusion in this review. Major practice guidelines, trial bibliographies, and pertinent reviews were examined to ensure the inclusion of relevant trials. The following section reviews data from pivotal trials to review the efficacy of S-ICD for the termination of VT/VF. The S- ICD system consists of a pulse generator positioned over the sixth rib between the midaxillary and anterior axillary line and a tripolar parasternal electrode with the proximal and distal sensing electrodes positioned adjacent to the xiphoid process and manubriosternal junction, respectively. The conversion of the efficacy of the S-ICD after the first shock ranges from 88%-90.1% and 98.2%-100% after 5 shocks based on the current evidence. The device also has a 99% complication-free rate at 180 days with no increased complications even in ESRD patients. The PRAETORIAN trial showed non-inferiority of the S-ICD to transvenous ICD concerning device-related complications or inappropriate shocks in patients. S-ICD has several advantages including no need for fluoroscopy for implantation, decreased risk of infections, complications, and evidence of safety even in high-risk populations like ESRD. The limitations include the inherent lack of pacing abnormalities, the increase in inappropriate shocks compared to transvenous ICD, and non-reliability if there are baseline T wave abnormalities, especially in the inferior leads. Thus, S-ICD can be considered as an alternative to transvenous ICD in patients with an indication for defibrillator therapy but with no indication for pacing.
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Desfibriladores Implantáveis , Falência Renal Crônica , Taquicardia Ventricular , Arritmias Cardíacas/etiologia , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis/efeitos adversos , Humanos , Falência Renal Crônica/etiologia , Taquicardia Ventricular/terapia , Resultado do TratamentoRESUMO
The leading cause of preventable death in the world is smoking; leading to 7 million deaths attributable to tobacco use per year worldwide and 480,000 deaths per year in the United States. Though the actual rates of smoking in the United States have been on the decline, the advent of electronic cigarettes (E cigs) in 2007 was met with immense reception especially among the youth. Though initially thought to be a means to facilitate smoking cessation, recent evidence and the E cigs epidemic suggest its implications in significant morbidity and mortality. The other alternatives for conventional tobacco cigarettes have also been implicated in cardiovascular diseases. This manuscript aims to review E cigarettes and other alternatives to conventional cigarettes, and their impact on cardiovascular health. We conducted a Medline search using various combinations of "Cigarettes," "E cigarettes," "Smokeless tobacco," "hookah", and "cardiovascular risk" to identify pivotal trials published before May 10, 2020, for inclusion in this review. Concurrently, major practice guidelines, trial bibliographies, and pertinent reviews were examined to ensure inclusion of relevant trials. A consensus among the authors was used to choose items for narrative inclusion. The following section reviews data from pivotal trials to review the effects of E cigarettes, smokeless tobacco, hookah, nicotine replacement therapy on cardiovascular mortality and morbidity. There are many alternatives to conventional tobacco cigarette smoking, but none can be attributed as absolutely safe from a cardiovascular health perspective. The abundance of evidence regarding its impact on cardiovascular mortality and morbidity does not position it as a safe alternative, but an alternative means of smoking nicotine. The humongous rise in popularity and its gain in favor among the younger population poses a serious threat to the cardiovascular well-being of the exposed. Thus, E cigs and other alternatives of cigarette smoking do impart differing risks in cardiovascular mortality and morbidity, with the possible exception of nicotine replacement therapy.
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Sistemas Eletrônicos de Liberação de Nicotina , Abandono do Hábito de Fumar , Produtos do Tabaco , Sistemas Eletrônicos de Liberação de Nicotina/estatística & dados numéricos , Humanos , Fumar/epidemiologia , Abandono do Hábito de Fumar/estatística & dados numéricos , Dispositivos para o Abandono do Uso de Tabaco , Estados Unidos/epidemiologiaRESUMO
Invasive coronary angiography (ICA) serves as a very important tool in the diagnosis of coronary artery disease (CAD) and provides information for further intervention. Fractional Flow Reserve (FFR) at the time of ICA is the gold standard to analyze the hemodynamic and physiologic significance of moderate coronary stenosis. The dawn of coronary CT angiography (CTA) has helped in visualizing the anatomy of coronary arteries. Computed Fractional Flow Reserve (FFRCT) from such an imaging study shows promise in providing valuable data about physiology on top of the anatomy noninvasively; which can guide decision-making process for revascularization. This manuscript aims to review the accuracy of FFRCT obtained from a coronary CTA in the diagnosis of hemodynamically significant coronary artery stenosis and ruling out nonsignificant coronary artery stenosis when compared to the Gold standard of FFR obtained during ICA. We conducted a Medline search using various combinations of "FFRCT," "ICA" "noninvasive," "significant stenosis," and "CAD" to identify pivotal randomized trials published before May 1, 2020, for inclusion in this review. Concurrently, major practice guidelines, trial bibliographies, and pertinent reviews were examined to ensure inclusion of relevant trials. A consensus among the authors was used to choose items for narrative inclusion. The following section reviews data from pivotal trials to determine a noninvasive strategy in appropriate patients to accurately detect functionally significant stenosis. For these trials, the sensitivity, specificity, and accuracy are compared. Trials reviewed: CTA, FFRCT, ICA, CT-myocardial perfusion imaging. FFRCT is a novel noninvasive modality which localizes significant "ischemia-causing" stenosis (≤0.80) by means of crystal fluid dynamics eliminating the need for vasodilators. The analysis of FFRCT by DISCOVER FLOW, DeFACTO, NXT trials revealed high sensitivity, negative predictive value, and good accuracy. The ADVANCE registry showed significantly lower events of CV death or myocardial infarction with a negative FFRCT (>0.80 study). The PLATFORM trial showed significant reduction in negative ICA with negative FFRCT, thus ultimately reducing the number of unnecessary percutaneous coronary intervention. Decrease in healthcare costs was noted with FFRCT, decreasing downstream testing, and invasive procedures. FFRCT is a novel modality for analyzing significant stenosis in CAD noninvasively. The high sensitivity of this modality could make it a good rule out tool to avoid unnecessary intervention in physiologically insignificant lesions. Limitations of this modality include low specificity, double exposure to contrast, turnaround time, and upfront costs. Further query into this matter is warranted.
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Doença da Artéria Coronariana , Reserva Fracionada de Fluxo Miocárdico , Angiografia por Tomografia Computadorizada , Angiografia Coronária , Doença da Artéria Coronariana/diagnóstico por imagem , Vasos Coronários/diagnóstico por imagem , Hemodinâmica , Humanos , Valor Preditivo dos Testes , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios XRESUMO
Trigger factors such as earthquakes, war, and terrorism have been shown to increase the risk of cardiovascular events in different studies. Similarly, strong emotions and psychological stress have been associated with myocardial infarction, symptomatic arrhythmias, and sudden cardiac death. Die-hard soccer, rugby, football, and baseball fans seem to be at risk of cardiac events, particularly in individuals with prior history of coronary artery disease. Transient hemodynamic changes, endothelial dysfunction, and an overwhelming sympathetic nervous system stimulation appear to affect cardiac hemostasis creating a procoagulant and arrhythmogenic environment. High-risk behaviors such as tobacco abuse and binge drinking appear to contribute to this risk generating a proinflammatory state characterized by elevated levels of endothelin-1 and overexpression of sCD40L, sVCAM-1, MCP-1, and TNF-alpha. The outcome of the game and unexpected results, especially among fans of the defeated team, seem to further correlate with adverse cardiovascular effects.
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Arritmias Cardíacas , Morte Súbita Cardíaca , Esportes , Humanos , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/etiologia , Futebol Americano , Futebol , Estresse Psicológico , Rugby , BeisebolRESUMO
de Winter's sign was first described by de Winter et al. in 2008 as a new electrocardiographic (EKG) pattern of acute proximal left anterior descending coronary artery (LAD) occlusion. Instead of the normal presentation of ST elevation, it is described as depression of ST segment at the J point in the precordial leads V1-V6, which are upsloping leading to hyper-acute T waves, with ST elevation in aVR. The initial retrospective systematic analysis proved this sign to be present in about 2% of anterior myocardial infarction. This review aims to address the important question of mode and urgency of intervention, on detection of de Winter's sign. In this review, we take a look at the de Winter's sign EKG characteristics, accuracy in diagnosis, typical patient presentation, and the outcomes of early intervention. We conducted a Medline search using various combinations of "de Winter's sign," "STEMI equivalent," "cardiac catheterization," and "thrombolysis" to identify pivotal research articles published before June 1, 2019, for inclusion in this review. Concurrently, major practice guidelines, trial bibliographies, and pertinent reviews were examined to ensure inclusion of relevant trials. A consensus among the authors was used to choose items for narrative inclusion. The following section reviews data from pivotal trials to determine the need for early invasive management in de Winter's sign. Research articles reviewed evaluating cardiac catheterization in de Winter's sign. de Winter's sign, although rare (~2%), should be promptly recognized, as it reveals underlying severe coronary artery pathology, frequently involving the LAD which is associated with a high rate of mortality. This systematic review emphasizes awareness and strong consideration of early activation of the cardiac catheterization lab with PPCI; which may yield better treatment outcomes. The evidence suggests that de Winter's sign, presenting with ST depression and T wave elevation, should indeed be treated as ST-elevation myocardial infarction (STEMI) equivalent, with prompt recognition and early intervention.
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The prevalence of heart failure (HF) has been on the rise with associated increase in hospitalizations, morbidity and mortality. These hospitalizations have led to increasingly more cost on and decreased quality of life for patients. CardioMEMS is one of the newer devices designed to help tackle this issue by allowing for better monitoring of HF patients. This device also allows for accurate recording of pulmonary artery pressures (PAPs) and has also been applied in various other areas, such as aneurysmal tears, for monitoring pressures. In this manuscript we will review the current state of CardioMEMS HF system and investigate some of the other areas for its promising use in the field.
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Atrial fibrillation (AF) poses a major health concern in the United States by affecting over 5 million people accounting for at least 15% to 25% of strokes. It can be asymptomatic or subclinical with its first presentation being stroke in 18%, and AF being only detected at the time of stroke. With evidence of subclinical AF associated with increased risk of ischemic stroke, recent developments indeed point towards wearables, especially smart watches, being quite effective and representing a novel method for screening for silent AF in the general population, and thereby reducing mortality and morbidity associated with it. This manuscript aims to review whether the photoplethysmography (PPG) technology, employed in the wearables to monitor heart rate, is accurate enough to aid in the diagnosis of AF that may remain asymptomatic or paroxysmal. It also explores the option of actually employing this method in the general population, the feasibility of this mode of diagnosis, sensitivity and specificity of this method compared to the conventional electrocardiogram (EKG), and the actual follow up with a practitioner and subsequent treatment of AF, if diagnosed. We conducted a Medline search using various combinations of "smart watch" "atrial fibrillation" "wearables", and "Kardia" to identify pivotal randomized trials published before June 1, 2019, for inclusion in this review. Concurrently, major practice guidelines, trial bibliographies, and pertinent reviews were examined to ensure inclusion of relevant trials. A consensus among the authors was used to choose items for narrative inclusion. The following section reviews data from pivotal trials to determine the effectiveness of smart watch technology in detecting AF in the general population. Trials reviewed evaluated apple watch, Kardia, Samsung wearables in diagnosis of AF. The fact that there is an increase in consumer use of wearables, smart devices, which can serve as health monitoring devices that can be used as a non-invasive, ambulatory assessment of heart rate and rhythm, is definitely novel. Intermittent short EKG recordings repeated over a longer-term period produced significantly better sensitivity for AF detection, with 4 times as many cases diagnosed compared with a single time-point measurement. Since there are limitations and further research into this new field is required, the wearable technology may not serve as the ultimate tool for diagnosis of AF, rather a nidus for the general population to seek medical advice for confirmation on being notified of having an irregular rhythm leading to prevention of morbidity and mortality associated with it.
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Valve replacement in high-risk patients with severe aortic stenosis has undergone a huge paradigm shift in the recent years in terms of procedural details and vascular access site for patients who have poor peripheral access. Carotid artery is one of the more promising access sites which has been proven to provide a good alternative site with comparable outcomes to transfemoral approach. In this manuscript, we will provide a review of the current literature on transaortic, transapical, transaxillary and transcarotid approaches to transcatheter aortic valve replacement (TAVR) while focusing on the transcarotid approach.
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One of the most important variables in assessing hemodynamic status in the intensive care unit (ICU) is the cardiac function and blood pressure. Invasive methods such as pulmonary artery catheter and arterial line allow monitoring of blood pressure and cardiac function accurately and reliably. However, their use is not without drawbacks, especially when the invasive nature of these procedures and complications associated with them are considered. There are several newer methods of noninvasive and minimally invasive hemodynamic monitoring available. In this manuscript, we will review these different methods of minimally invasive and non-invasive hemodynamic monitoring and will discuss their advantages, drawbacks and limitations.
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Out-of-hospital cardiac arrest (OHCA) is the leading cause of death in the United States, as 90% of them are fatal per the 2018 American Heart Association statistics. As many as fifty-percent of cardiac arrest events display an initial rhythm of pulseless ventricular tachycardia (pVT) and ventricular fibrillation (VF), and of those, coronary artery disease (CAD) is found in 60-80% of patients. Following return of spontaneous circulation, patients who present with ST-elevation myocardial infarction (STEMI) should undergo an early invasive strategy and primary intervention, which is well-established guideline-based management. The support of such a strategy in patients suspected to have underlying cardiac cause but without ST-elevation has been waxing and waning in the literature. The Coronary Angiography after Cardiac Arrest (COACT) trial was designed to compare survival between an immediate or delayed coronary angiography strategy in non-STEMI (NSTEMI) OHCA patients, following successful resuscitation. We present a systematic review of the history of management strategies in OHCA and propose guidelines to manage such patients in light of the COACT trial.