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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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.

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...

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.

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 ...

Myriad manifestations of Williams syndrome.

4 months male child presented with failure to thrive. On general examination child had normal O2 saturation with characterstic elfin facies. Further evaluation of the patient showed major manifestations of Williams syndrome in form of supravalvar aortic stenosis, branched pulmonary artery stenosis along with cardiomyopathy. Although the entity is known, this article shows comprehensive diagnostic workup with the aid of multimodality imaging techniques. The genetic diagnosis of Williams syndrome was confirme...

A Long-term Retrospective Evaluation of Functional and Radiographic Outcomes of Pediatric Hip Surgery in Hurler Syndrome.

After successful hematopoietic stem cell transplantation, maintaining function and mobility have become key goals in the management of patients with Hurler syndrome, (mucopolysaccharoidosis type 1H). The aim of this study was to establish the functional and radiologic outcomes after hip surgery in patients with this condition who had reached skeletal maturity.

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.

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.

Increased aortic tortuosity indicates a more severe aortic phenotype in adults with Marfan syndrome.

Patients with Marfan syndrome (MFS) have a highly variable occurrence of aortic complications. Aortic tortuosity is often present in MFS and may help to identify patients at risk for aortic complications.

Patients' Decision Making About Undergoing Transcatheter Aortic Valve Implantation for Severe Aortic Stenosis.

Aortic stenosis is the most common valve disease in Western countries, and its prevalence is increasing because of the aging population. Some patients, denied surgery because of high risk, can be offered transcatheter aortic valve implantation (TAVI). These patients are old and have comorbidities, and it is not always easy for them to make the decision about accepting TAVI.

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.

Association of Low-Density Lipoprotein Cholesterol-Related Genetic Variants With Aortic Valve Calcium and Incident Aortic Stenosis.

Plasma low-density lipoprotein cholesterol (LDL-C) has been associated with aortic stenosis in observational studies; however, randomized trials with cholesterol-lowering therapies in individuals with established valve disease have failed to demonstrate reduced disease progression.



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