PubMed Journals Articles About "Aortic Stenosis Patient With Hurler Syndrome After Bone" RSS

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Showing "Aortic stenosis patient with Hurler syndrome after bone" PubMed Articles 1–25 of 21,000+

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Progressive supra-aortic stenosis in a young adult with the findings of Singleton Merten Syndrome.

Singleton Merten Syndrome is an autosomal dominant disorder of unknown origin. Patients often present with muscular weakness, failure to thrive, abnormal dentition, glaucoma, psoriatic skin lesions, aortic calcification and musculoskeletal abnormalities. In this case, we present a young girl with a history of aortic root replacement, who had an unusual progressive supra-aortic stenosis managed with urgent surgery during the course of the syndrome. Cardiovascular involvement needs special attention, since it...

Fetal intervention for critical aortic stenosis: advances, research and postnatal follow-up.

Fetal aortic valvuloplasty is intended to alter the natural history of aortic stenosis evolving to hypoplastic left heart syndrome. The most recently reported data and advances on this procedure were reviewed.

Effects of Transcutaneous Aortic Valve Implantation on Aortic Valve Disease-Related Hemostatic Disorders Involving von Willebrand Factor.

Aortic valve stenosis (AVS) can be complicated by bleeding associated with acquired type 2A von Willebrand syndrome. The association of AVS and gastrointestinal bleeding from angiodysplasia is defined as Heyde syndrome. We sought to evaluate the effect of transcutaneous aortic valve implantation (TAVI) on hemostasis disorders and to assess its effectiveness to treat Heyde syndrome.

Aortic arch atherosclerosis in patients with severe aortic stenosis can be argued by greater day-by-day blood pressure variability.

Although it is well known that the prevalence of aortic arch plaques, one of the risk factors for ischemic stroke, is high in patients with severe aortic stenosis, the underlying mechanisms are not well understood. Increased day-by-day blood pressure (BP) variability is also known to be associated with stroke; however, little is known on the association between day-by-bay BP variability and aortic arch atherosclerosis in patients with aortic stenosis. Our objective was to clarify the association between day...

Transcatheter management of neonatal aortic stenosis.

Neonatal aortic valvar stenosis can be challenging to treat because of the varied morphology of the valve, the association with hypoplasia of other left heart structures, and the presence of left ventricular systolic dysfunction or endomyocardial fibroelastosis. Balloon valvuloplasty and surgical valvotomy have been well described in the literature for the treatment of neonatal aortic stenosis. Transcatheter therapy for neonatal aortic stenosis is the preferred method at many centres; however, some centres ...

Transcatheter, valve-in-valve transapical aortic and mitral valve implantation, in a high risk patient with aortic and mitral prosthetic valve stenoses.

Transcatheter valve implantation continues to grow worldwide and has been used principally for the nonsurgical management of native aortic valvular disease-as a potentially less invasive method of valve replacement in high-risk and inoperable patients with severe aortic valve stenosis. Given the burden of valvular heart disease in the general population and the increasing numbers of patients who have had previous valve operations, we are now seeing a growing number of high-risk patients presenting with pros...

Low Gradient Aortic Stenosis.

Severe low-gradient (LG) aortic stenosis (AS) [aortic valve area (AVA) ≤ 1.0 cm(2), mean pressure gradient (MG) 

Complications Associated With Nitrate Use in Patients Presenting With Acute Pulmonary Edema and Concomitant Moderate or Severe Aortic Stenosis.

We evaluate the incidence of complications associated with the use of nitrates in patients presenting with acute pulmonary edema and concomitant moderate or severe aortic stenosis compared with patients without aortic stenosis. Nitrates are contraindicated in severe aortic stenosis because of the theoretical yet unproven risk of precipitating profound hypotension.

Anaesthesia-related haemodynamic complications in Williams syndrome patients: A review of one institution's experience.

Williams syndrome is a genetic disorder associated with cardiac pathology, including supravalvular aortic stenosis and coronary artery stenosis. Sudden cardiac death has been reported in the perioperative period and attributed to cardiovascular pathology. In this retrospective audit, case note and anaesthetic records were reviewed for all confirmed Williams syndrome patients who had received an anaesthetic in our institution between July 1974 and November 2009. There were a total of 108 anaesthetics adminis...

18F-FDG Uptake and Calcifications on Positron Emission Tomography/Computed Tomography in octogenarians with severe aortic stenosis.

The degree of inflammation within the atherosclerotic plaque can be detect non--invasively by positron emission tomography (PET) with 18F--fluorodeoxyglucose (18F--FDG). The incidence of aortic plaques with 18F--FDG increased uptake in octogenarians with aortic stenosis is unknown. Aim of this study was to evaluate the frequency of inflamed aortic atherosclerotic plaques in octogenarians with or without severe aortic stenosis and their correlations with calcifications.

What is the ultimate test that lowering lipoprotein(a) is beneficial for cardiovascular disease and aortic stenosis?

Lipoprotein(a) [Lp(a)] is a risk factor for cardiovascular disease (CVD) and calcific aortic valve stenosis. We review recent studies that highlight Lp(a) in CVD and calcific aortic valve stenosis and propose pathways to clinical registration of Lp(a)-lowering agents.

High left ventricular outflow tract gradient: Aortic stenosis, obstructive hypertrophic cardiomyopathy or both?

The authors report the case of a patient diagnosed with both hypertrophic cardiomyopathy and aortic stenosis. Due to clinical deterioration, additional investigation was performed, and a high left ventricular outflow tract gradient was identified. Correct identification of the condition causing the symptoms was challenging, and involved several imaging techniques, the contribution of transesophageal echocardiography being crucial. The final diagnosis of severe aortic stenosis led to successful valve replace...

Transcatheter aortic valve replacement: When invasive surgery is not an option.

Aortic stenosis is a common problem that typically results from calcification and degenerative changes of the aortic valve that occur with advancing age. Because medical therapies have no clinically proven role, the management of severe aortic stenosis has primarily been surgical. For patients who are too high-risk for surgical aortic valve repair, transcatheter aortic valve repair (TAVR), approved by the FDA in 2011, may be an option. This article outlines what clinicians need to know about TAVR.

The left ventricle in aortic stenosis - imaging assessment and clinical implications.

Aortic stenosis has an increasing prevalence in the context of aging population. In these patients non-invasive imaging allows not only the grading of valve stenosis severity, but also the assessment of left ventricular function. These two goals play a key role in clinical decision-making. Although left ventricular ejection fraction is currently the only left ventricular function parameter that guides intervention, current imaging techniques are able to detect early changes in LV structure and function even...

Early and mid-term results of isolated aortic valve replacement for aortic stenosis in octogenarians.

As transcatheter aortic valve implantation (TAVI) may become a potential treatment for high-risk patients with aortic stenosis (AS), evaluation of outcomes after open aortic valve replacement (AVR) in elderly patients is warranted. We documented early and late outcomes after isolated AVR in octogenarians compared with younger age groups.

High-Risk Patients With Inoperative Aortic Stenosis: Use of Transapical, Transaortic, and Transcarotid Techniques.

Patient characteristics and procedural outcomes from nontransfemoral (non-TF) transcatheter aortic valve replacement (TAVR) in high-risk or inoperable patients with aortic stenosis have been incompletely reported. The purpose of this study was to compare outcomes with non-TF TAVR access techniques including transapical (TA), transaortic (TAo), and transcarotid (TC) TAVR with a balloon-expandable valve.

Treatment of Acquired von Willebrand Syndrome in Aortic Stenosis With Transcatheter Aortic Valve Replacement.

This study sought to investigate the prevalence of abnormal von Willebrand multimers (AbM) in patients undergoing transcatheter aortic valve replacement (TAVR) and the impact of TAVR on the underlying factor variances.

Circulating Levels of Matrix Gla Protein and Progression of Aortic Stenosis: A Substudy of the Aortic Stenosis Progression Observation: Measuring Effects of Rosuvastatin (ASTRONOMER) Trial.

Matrix γ-carboxyglutamate protein is an inhibitor of cardiovascular calcification. The objective of this substudy of the Aortic Stenosis Progression Observation: Measuring Effects of Rosuvastatin (ASTRONOMER) trial was to examine the relationship between total (ie, carboxylated [active] form + uncarboxylated [inactive] form) circulating desphosphorylated matrix γ-carboxyglutamate protein (dpMGP) level and the progression rate of aortic stenosis (AS).

Coronary microvascular dysfunction and aortic stenosis: An update.

The coronary microcirculatory impairment is a key feature of the pathophysiology of aortic stenosis (AS), the most operated valvular disease over the world. Several studies showed this coronary microcirculatory impairment in AS, using different tools and protocols, in various patient population of AS. This article will review the impairment of the coronary microcirculation in AS underlining its multifactorial origin, its functional part related to the hemodynamic consequences of AS, its complex relationship...

Coronary artery disease and transcatheter aortic valve replacement: current treatment paradigms.

Aortic stenosis is the most common form of valvular heart disease in the elderly population and is often diagnosed in individuals who also have coronary artery disease. Surgical aortic valve replacement has been the standard of care for the treatment of aortic stenosis during the past decades, but the availability of transcatheter aortic valve replacement has now allowed different options for high or extreme surgical risk patients. The management of coronary artery disease in patients undergoing transcathet...

Usefulness of global left ventricular longitudinal strain for risk stratification in low ejection fraction, low-gradient aortic stenosis: results from the multicenter true or pseudo-severe aortic stenosis study.

The objective of this study was to examine the impact of left ventricular (LV) global longitudinal strain (GLS) measured at rest and at dobutamine stress echocardiography on the outcome of patients with low LV ejection fraction and low-gradient aortic stenosis.

Cardiac Tamponade After Transcatheter Aortic Valve Replacement Using a Transaortic Approach.

Transcatheter aortic valve replacement (TAVR) is considered an option for patients with severe aortic stenosis who are not surgical candidates. We describe the case of a patient who presented with cardiac tamponade shortly after uneventful TAVR by a direct aortic approach. The patient was brought to the operating room for exploration and repair. Although TAVR is less invasive than traditional open aortic valve replacement, TAVR nonetheless poses serious risk. This case highlights a potential complication of...

Transcatheter Aortic Valve Replacement for Patients With Aortic Valve Stenosis Complicated With Moyamoya Disease.

Moyamoya disease (MMD) is a rare disease characterized by occlusive intracranial arteriopathy with formation of abnormal cerebrovascular collateral networks. Conventional cardiovascular surgical procedures using cardiopulmonary bypass for patients with MMD is challenging because low cerebral perfusion pressure and nonpulsatile (continuous) flow during cardiopulmonary bypass can cause severe cerebral ischemia. We successfully performed transcatheter aortic valve replacement in 3 women with severe aortic valv...

Preprocedural CT Evaluation of Transcatheter Aortic Valve Replacement: What the Radiologist Needs to Know.

Aortic valve stenosis is the most common valvular heart disease in the Western world. When symptomatic, aortic valve stenosis is a debilitating disease with a dismal short-term prognosis, invariably leading to heart failure and death. Elective surgical valve replacement has traditionally been considered the standard of care for symptomatic aortic valve stenosis. However, several studies have identified various subgroups of patients with a significantly elevated risk for surgery-related complications and dea...

Experiences of and Coping With Severe Aortic Stenosis Among Patients Waiting for Transcatheter Aortic Valve Implantation.

Aortic stenosis (AS) is the most common valve disease in Western countries. Transcatheter aortic valve implantation (TAVI) has made it possible to treat patients with higher surgical risks. These patients are informed about their poor prognosis with only months or a few years to live without treatment. Because of their severe symptoms, limitations, and suffering, patients awaiting TAVI need special attention.

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