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

18:35 EDT 1st April 2015 | BioPortfolio

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Showing "Aortic stenosis patient with Hurler syndrome after bone" PubMed Articles 1–25 of 22,000+

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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.

Endovascular Treatment for Primary Aortic Angiosarcoma to Relieve Thoracic Aortic Stenosis.

Aortic aneurysms and stenosis are widely treated with endovascular procedures in the current era. In this report, we present endovascular stent grafting for symptomatic treatment of severe thoracic aortic stenosis caused by an aortic sarcomatoid carcinoma in a 73-year-old female. The peri- and post-operative courses were complicated with tumor embolisms that were managed both endovascular and surgical measures.

The impact of the metabolic syndrome on the outcome after aortic valve replacement.

The aim of this study was to examine the influence of the metabolic syndrome on the left ventricular geometry as well as on the early and mid-time outcome in patients with aortic stenosis who underwent aortic valve replacement.

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 ...

97 Value of Cardiopulmonary Exercise and Non-invasive Haemodynamic assessment during Exercise in Patients with Asymptomatic Severe Aortic Stenosis.

Timing of aortic valve replacement in patients with severe asymptomatic aortic stenosis (VD) is controversial. Exercise testing may uncover symptoms and early cardiac dysfunction. However there is little information on the value of cardiopulmonary exercise (CPX) testing combined with haemodynamic assessment during exercise in this patient group. We report preliminary results of CPX and haemodynamic data in this patient cohort.

Low Gradient Aortic Stenosis.

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

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.

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.

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.

High-sensitivity troponin I concentrations are a marker of an advanced hypertrophic response and adverse outcomes in patients with aortic stenosis.

High-sensitivity cardiac troponin I (cTnI) assays hold promise in detecting the transition from hypertrophy to heart failure in aortic stenosis. We sought to investigate the mechanism for troponin release in patients with aortic stenosis and whether plasma cTnI concentrations are associated with long-term outcome.

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.

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...

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...

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.

Coronary Blood Flow in Patients With Severe Aortic Stenosis Before and After Transcatheter Aortic Valve Implantation.

Patients with severe aortic stenosis and no obstructed coronary arteries are reported to have reduced coronary flow. Doppler evaluation of proximal coronary flow is feasible using transesophageal echocardiography. The present study aimed to assess the change in coronary flow in patients with severe aortic stenosis undergoing transcatheter aortic valve implantation (TAVI). The left main coronary artery was visualized using transesophageal echocardiography in 90 patients undergoing TAVI using the Edwards SAPI...

Direct aortic transcatheter valve implantation in a porcelain aorta.

Transcatheter aortic valve implantation has been designed to treat elderly patients with severe aortic stenosis at high risk for surgery, and is generally performed retrogradely with vascular access. However, in certain patients, this access is either not possible or deemed to carry a high risk of vascular injury. We report our experience of a direct aortic approach in a 78-year old man with severe aortic stenosis, excluded from standard aortic valve replacement due to a porcelain aorta, and affected by sev...


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