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

14:50 EDT 5th October 2015 | BioPortfolio

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Showing "Aortic stenosis patient with Hurler syndrome after bone" PubMed Articles 1–25 of 20,000+

Osseous and chondromatous metaplasia in calcific aortic valve stenosis.

Aortic valve replacement for calcific aortic valve stenosis is one of the more common cardiac surgical procedures. However, the underlying pathophysiology of calcific aortic valve stenosis is poorly understood. We therefore investigated the histologic findings of aortic valves excised for calcific aortic valve stenosis and correlated these findings with their associated clinical features.

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 ...

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.

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...

Severe aortic valve stenosis in the elderly: high prevalence of sleep-related breathing disorders.

Aortic valve stenosis is common in the elderly, with a prevalence of nearly 3% in patients aged 75 years or older. Despite the fact that sleep-related breathing disorders (SRBD) are thought to be associated with cardiac disease, little is known about their prevalence in this patient cohort. The purpose of this study was to evaluate the prevalence of SRBD in older patients with aortic valve stenosis admitted for transcatheter aortic valve implantation.

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.

Patient-defined goals for the treatment of severe aortic stenosis: a qualitative analysis.

Patients with severe aortic stenosis (AS) at high risk for aortic valve replacement are a unique population with multiple treatment options, including medical therapy, surgical aortic valve replacement and transcatheter aortic valve replacement (TAVR). Traditionally, in elderly populations, goals of treatment may favour quality of life over survival. Professional guidelines recommend that clinicians engage patients in shared decision making, a process that may lead to decisions more aligned with patient-def...

Anesthetic management for craniotomy in a patient with massive cerebellar infarction and severe aortic stenosis: a case report.

Severe aortic stenosis combined with coronary heart disease remarkably increases the risk of perioperative morbidity and mortality during noncardiac surgery. Surgery and anesthesia often complicate the perioperative outcome if adequate monitoring and proper care are not taken. Therefore, understanding of the hemodynamic changes and anesthetic implications is an important for successful perioperative outcome. This report described the anesthetic management of a patient with a massive cerebellar infarction wh...

Determinants in treatment decision-making in older patients with symptomatic severe aortic stenosis.

Three treatment options are available for patients with aortic stenosis: surgical aortic valve replacement (SAVR), transcatheter aortic valve implantation (TAVI) and medical treatment (MT). However, little is known about how Heart Team treatment decisions are made under routine conditions. The aim of this study was to identify the cardiac and geriatric components associated with treatment decision-making in older patients with symptomatic severe aortic stenosis.

Expanding the phenotype of feingold syndrome-2.

Feingold syndrome-2 has been recently shown to be caused by germline heterozygous deletions of MIR17HG with 10 reported patients to date. Manifestations common to both Feingold syndrome-1 and Feingold syndrome-2 include microcephaly, short stature, and brachymesophalangy; but those with Feingold syndrome-2 lack gastrointestinal atresias. Here we describe a 14-year-old male patient who presented to our Cardiovascular Genetics Clinic with a history of a bicuspid aortic valve with aortic stenosis, short statur...

Aortic valve decalcification for severe aortic valve stenosis in the elderly: medium-term results.

To present the results of a novel technique of aortic valve decalcification (AVD) in a consecutive population of elderly patients with severe aortic valve stenosis (AVS) and small aortic annulus.

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...

Efficacy and Safety of Transcatheter Aortic Valve Implantation in Aortic Stenosis Patients With Extreme Age.

To investigate the in-hospital and long-term outcomes of patients at extreme age with severe symptomatic aortic stenosis (AS) who underwent transcatheter aortic valve implantation (TAVI).

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...

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.

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...


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